Step Up To Medicine - Neurology

Front 1

Epidemiology of ischemic stroke/cerebral infarction

Back 1

- stroke or CVA is the 3rd most common cause of death in the US
- it is the leading cause of neurologic disability

Front 2

Classes of ischemic stroke

Back 2

1. transient ischemic attack (TIA) - neurologic deficit lasting from a few minutes to no more than 24 hours (usually less than 30 minutes)
2. Reversible ischemic neurologic deficit- the same as TIA except for the duration of symptoms-lasts for 24 hours to 2 weeks- not commonly used term
3. evolving stroke- stroke that is worsening
4. completed stroke- maximal deficit has already occurred

Front 3

Transient ischemic attack

Back 3

1. a neurologic deficit that lasts from a few minutes to no more than 24 hours (but usually less than 30 minutes)
- stroke may be indistinguishable from a TIA initially. the duration of symptoms is the determining difference.
- symptoms are transient because reperfusion occurs, either because of collateral circulation or because of the breaking up of an embolus
- the blockage of blood flow does not last long enough to cause permanent damage
- usually embolic. however, transient hypotension in the presence of severe carotid stenosis (>75% occlusion) can lead to TIA
- Once the patient has a TIA, there is a high risk of stroke in subsequent months (30% 5-year risk). Look for cardiac factors

Front 4

Risk factor for ischemic strokes (which are the most important RFs)
- what are the most important RFs in young patients?

Back 4

1. the most important risk factors are AGE and HTN
2. other risk factors include- smoking, DM, hyperlipidemia, Afib, CAD, fhx, previous CVA/TIA and carotid bruits
3. OCP use, hypercoagulable states (protein C and S deficiency, APA syndrome), vasoactive drug use (cocaine, amphetamines), polycythemia vera and sickle cell disease

Front 5

Causes of ischemic stroke
1. embolic - from where?
2. Thrombotic
3. Lacunar
4. other

Back 5

1. Embolic stroke is the most common. Heart is the most common source, usually due to embolization of a mural thrombus in patients with Afib. Other sources include: internal carotid artery, aorta, paradoxical
2. thrombotic- atherosclerotic lesions may be in the large arteries of the neck (carotid artery disease, which most commonly involves the bifurcation of the common carotid), or in the medium sized arteries in the brain - especially the MCA
3. lacunar stroke- small vessel thrombotic disease - causes approximately 20% of all strokes, usually affects subcortical structures (basal ganglia, thalamus, internal capsule, and brainstem)-- not cerebral cortex. HTN is major predisposing factor (80-90% of the time it is present). Diabetes also a big one. Narrowing of the arterial lumen is due to thickening of the vessel wall - not thrombosis. Arteries affected include small branches of the MCA, arteries of circle of willis, and basilar and vertebral arteries. When the vessels occlude, small infarcts result, and when they heal they are called lacunes
4. other- low cardiac output and anoxia (may cause global ischemia and infarction)

Front 6

Clinical features of....
1. thrombotic stroke
2. embolic stroke

Back 6

1. the onset of symptoms may be rapid or stepwise. Classically, the patient awakens from sleep with the neurologic deficits
2. the onset of symptoms is very rapid (within seconds) and the deficits are MAXIMAL initially. Symptoms depend on the site of occlusion. MCA is most commonly affected--> contralateral hemiparesis and hemisensory loss. Aphasia if dominant hemisphere is involved- 90% of people are left dominant. Apraxia, contralateral body neglect, confusion if the non-dominant side is affected

Front 7

Clinical features of lacunar strokes-- where is affected if the following symptoms are present?
1. pure motor
2. pure sensory
3. ataxic hemiparesis
4. clumsy hand dysarthria

Back 7

1. pure motor- internal capsule
2. pure sensory - thalamus
3. ataxic hemiparesis-- incoordination ipsilaterally
4. pons

Front 8

Symptoms seen with:
1. an ACA stroke
2. MCA stroke
3. Vertebral/basilar stroke
4. lacunar (a. internal capsule, b. pons, c. thalamus)

Back 8

1. contralateral LE and face
2. aphasia, contralateral hemiparesis
3. ipsilateral: ataxia, diplopia, dysphagia, dysarthria and vertigo. Contralateral: homonymous hemianopsia with basilar/PCA lesions (visual field loss on the left or right side of vertical midline
4. internal capsule- pure motor hemiparesis, pons- dysarthria, clumsy hand, thalamus- pure sensory

Front 9

Diagnosis of stroke

Back 9

1. CT scan (without contrast) of head
- this differentiates between ischemic and hemorrhagic strokes and is the first imaging test that should be done. No contrast in case of hemorrhage. Ischemic strokes appear as dark areas, hemorrhagic look white
- it may take 24-48 hours to see an infarct, but it is useful in ruling out intracerebral hemorrhage (ICH)
2. MRI of brain- more sensitive than CT- identifies 95% of infarcts within 24 hours, but it takes longer to perform so it is not ideal for the ER
3. ECG- acute MI or Afib may be cause of embolic strokes
4. carotid duplex US- assess degree of stenosis if present
5. MRA - definitive test for identifying stenosis of the vessels of the head and neck or for aneurysms. Evaluates carotids, verterbobasilar circulation, circle of willis and anterior, middle and posterior cerebral arteries

Front 10

Complications of ischemic stroke

Back 10

1. progression of neurologic insult
2. cerebral edema- occurs within 1-2 days can cause mass effects for up to 10 days. hyperventilation and mannitol may lower ICP
3. hemorrhage into the infarction/hemorrhagic transformation is rare
4. seizures- less than 5% of patients

Front 11

Acute treatment of ischemic stroke

Back 11

Early recognition of causes is unreliable, and early treatment is critical.
a. tPA/thrombolytic- if administered within 3 hours of the onset of acute ischemic stroke, improved clinical outcome is seen at 3 months. Do NOT give tPA if the time of the stroke is unknown, if it has been more than 3 hours, or if the patient has uncontrolled HTN, bleeding disorder or is anticoagulated, or a history of recent trauma or surgery. -- increased risk of hemorrhagic transformation
- if give tPA- do NOT give aspirin and do frequent neuro checks x 24 hrs to look for signs of bleed. Keep BP < 185/100 mmHg
- elevate HOB to 30 deg and evaluate airway, keep NPO - prevent aspiration

Front 12

Use of aspirin in acute treatment of ischemic stroke

Back 12

1. Best when given within 24 hour of symptom onset
- do not give to a patient who receives thrombolytic therapy
- give if patient presents 3 or more hours after onset of sx
- if patient cannot tolerate aspirin, give clopidogrel/plavix, and if cannot take plavix then give ticlopidine
- anticoagulants like heparin and warfarin are NOT effective

Front 13

BP control in acute management of ischemic stroke

Back 13

1. in general do NOT give antihypertensives agents unless one of the following 3 situations is present
a. BP is very high ( systolic > 220, diastolic > 120, or MAP > 130)
b. significant medical indication for antihypertensive- like acute MI, aortic dissection, severe heart failure, hypertensive encephalopathy
c. the patient is receiving thrombolytic therapy- aggressive BP control to reduce risk of bleeding

Front 14

Prevention of ischemic stroke

Back 14

1. specific recs depend on risk factors
- control risk factors - HTN, DM, smoking, hypercholesterolemia, obesity
- give aspirin
- carotid endarterctomy- if symptomatic and > 70% stenosis. If asx - no surgery, just aspirin and RF reduction
2. for strokes due to embolism-- anticoagulation (aspirin), reduction of atherosclerotic RFs
3. for prevention of lacunar strokes-- control HTN

Front 15

Causes of Intracerebral Hemorrhage (ICH)

Back 15

1. HTN - particularly a sudden increase in BP - the most common cause (50-60% of cases)
- HTN causes a rupture of the small vessels deep within the brain parenchyma. Chronic HTN causes degeneration of the small arteries, leading to microaneurysms which rupture easily
- Typically seen in older patients, risk increases with age
2. ischemic stroke may convert to hemorrhagic stroke
3. other causes include amyloid angiopathy (10%), anticoagulant/thrombolytic use, brain tumors, AV malformations

Front 16

Locations of ICH

Back 16

1. basal ganglia (66%)
2. pons (10%)
3. Cerebellum (10%)
4. other cortical areas

Front 17

Clinical features of intracerebral hemorrhage (ICH)

Back 17

1. sudden onset of a focal neurologic deficit that worsens steadily over 30-90 minutes
2. altered level of consciousness, stupor, coma
3. headache, vomiting
4. signs of inc ICP

Front 18

Diagnosis of ICP

Back 18

1. CT scan of the head diagnoses 95% of ICH
2. Coag panel and platelets -- look for bleeding diathesis

Front 19

Complications of ICH

Back 19

1. Increased ICP
2. Seizures
3. Rebleeding
4. Vasospasm
5. hydrocephalus
6. SIADH- hyponatremia and hypervolemia

Front 20

Treatment of ICH

Back 20

- Admission to the ICU
- ABCs- airway management is crucial. Intubation often required
- BP reduction - elevated BP increases ICP and can cause further bleeding. However, hypotension can lower cerebral blood flow, worsening neuro deficits. Make sure to reduce BP gradually
- Treatment of BP is indicated if systolic > 160 - 180 or diastolic > 105. Treatment for lower BP is controversial
- Nitroprusside is the agent of choice
- Mannitol-- osmotic diuretic that can be used to decrease ICP
- NO steroids
- Rapid surgical decompression of cerebellar hematoms can be life saving to prevent tonsilar herniation and respiratory arrest, however surgery is NOT helpful in most cases of ICH

Front 21

General characteristics of subarachnoid hemorrhage
a. mortality rate
b. locations

Back 21

a. mortality rate is as high as 40-50% at 30 days
b. locations- saccular aneurysms occur at bifurcations of the arteries of the circle of willis

Front 22

Causes of subarachnoid hemorrhage

Back 22

1. most common - ruptured berry aneurysm- higher morbidity and mortality than other causes
2. trauma can be a common cause
3. AV malformation

Front 23

Clinical features of subarachnoid hemorrhage (SAH)

Back 23

1. sudden, severe (often excrutiating) headache in the absence of focal neurologic symptoms. Classically described as the "worst headache of my life" but may also be more subtle
2. sudden, transient LOC- in approx 50%
3. vomiting (common)
4. Meningeal irritation, nuchal rigidity, and photophobia - can take several hours to develop
5. death- 25-50% of patients die with the first rupture. Those who will recover regain consciousness within the first few minutes
6. retinal hemorrhages- up to 30%

Front 24

Diagnosis of subarachnoid hemorrhage

Back 24

1. non-contrast CT scan- identifies the majority of SAHs
2. Perform lumbar puncture if the CT scan is unrevealing or negative and there is a high clinical suspicion. Blood in the CSF is the hallmark feature. -- make sure it is not from a traumatic tap. Xanthrochromia (yellowish tint to CSF) implies that the blood has been there for awhile (had time to lyse) and is not due to a traumatic tap. Gold
standard for diagnosis
- After diagnosis, get a cerebral angiogram to locate source and plan for clipping

Front 25

Complications of subarachnoid hemorrhage

Back 25

1. re-rupture in 30% of patients
2. vasospasm- occurs in 50%- can lead to ischemia/infarct--> stroke
3. hydrocephalus (communicating) -- blood in CSF hinders CSF flow
4. seizures may occur (blood is an irritant)
5. SIADH

Front 26

Treatment of subarachnoid hemorrhage

Back 26

1. surgical- consult neurosurg. Clipping is main tx to prevent rebleeding
2. Medical- therapy reduces the risk of rebleeding and cerebral vasospasm- bed rest in a quiet, dark room. Stool softeners to avoid straining. Analgesia for HA (acetaminophen), IV fluids, control BP- lower pressure gradually as increased BP may be compensation for decreased cerebral perfusion pressure (secondary to increased ICP or cerebral arterial narrowing)
- calcium channel blocker (nifedipine) for vasospasm- lowers the incidence of cerebral infarction by 1/3

Front 27

Parkinson's disease
1. etiology
2. age of onset
3. diagnosis

Back 27

1. results from loss of dopamine-containing neurons- nerve cells that are located in the pigmented substantia nigra and the locus ceruleus in the midbrain
2. obset is usually after age 50
- parkinsonism refers to the symptoms and signs of parkinson's disease can result from many conditions (e.g. medications)
3. parkinson's disease is essentially a clinical diagnosis. lab studies play NO role

Front 28

clinical features of parkinson's disease

Back 28

1. pill-rolling tremor at rest (worsens with emotional stress). Tremor goes away when performing routine tasks-- unlike an intention tremor
2. bradykinesia - slowness of voluntary movements
3. rigidity is characteristic- "cogwheel rigidity"- ratchet -like jerking elicited by having the patient clench one fist and testing the opposite limb
4. poor postural reflexes- difficulty initiating first step. walking with small shuffling steps and a stooped posture
5. masked (expressionless) facies, decreased blinking
6. dysarthria and dysphagia, micrographia (small handwriting)
7. impairment of cognitive function (dementia in advanced disease)
8. autonomic dysfunction - orthostatic hypotension, constipation, increased sweating, and oily skin
9. personality changes early on- withdrawn apathetic, dependent. depression is common
10. follows a progressive course- significant disability presents with 5-10 years and indirectly leads to increased mortality

Front 29

Treatment of parkinson's disease

Back 29

1. No cure - goals are to delay disease progression and relieve symptoms
2. Carbidopa-Levadopa (Sinemet) = drug of choice for treating parkinsonian symptoms. SE = dyskinesias (involuntary, often choreic movements)- can occur after 5-7 yrs of treatment. Major concern and thus warrants waiting to use this treatment until all others have failed. n/v, anorexia, HTN, hallucinations. Levadopa shows an "on-off" phenomena- over the course of the day-- fluctuations in symptoms
3. dopamine receptor agonists- pramipexaole, bromocriptine - may control symptoms and delay need for levadopa. Initiate once diagnosis is made. Pramipexole is the agent of choice. Can be useful for sudden episodes of immobility "freezing"
4. selegiline - MOA inhibitor - increases dopamine and decreases metabolism of levadopa
5. amantadine- antiviral agent- mildly beneficial early on
6. anticholinergic drugs- trihexyphenidyl and benztropine- esp for patients with prominent tremor
7. amitriptyline - tricyclic
8. deep brain stimulation - if patient does not respond to meds or has severe disease before 40 yo

Front 30

Medications that cause parkinsonian symptoms

Back 30

1. neuroleptic drugs (chlorpromazine, haloperidol, perphenazine)
2. metoclopramide (reglan) - anti-emetic
3. reserpine

Front 31

tremor vs bradykinesia as a prognostic indicator in Parkinson's disease

Back 31

patients with a prominent tremor have a better prognosis than those who have bradykinesia predominantly

Front 32

Progressive Supranuclear Palsy (PSP)

Back 32

- degenerative condition of the brainstem, basal ganglia, and cerebellum -- most commonly in middle-aged to elderly men
- like parkinson's disease, PSP causes bradykinesia, limb rigidity, cognitive decline and follows a progressive course
- unlike parkinson's disease though, PSP does NOT cause tremor, and does cause ophthalmoplegia

Front 33

Subclavian Steal Syndrome

Back 33

- caused by stenosis of subclavian artery proximal to the origin of the vertebral artery-- exercise of the left arm causes reversal of blood flow down the ipsilateral vertebral artery to fill the subclavian artery distal to the stenosis. This leads to decreased cerebral perfusion-- blood stolen from the basilar system
- BP in the left arm is lower than in the right arm-- decreased pulse in the left arm
- upper extremity claudication
- Treatment- surgical bypass

Front 34

What is the most common location of a CVA and what are the symptoms?

Back 34

MCA- middle cerebral artery. Symptoms include contralateral weakness, sensory loss and hyperreflexia

Front 35

Who should be screened by carotid duplex US?

Back 35

All patients with a carotid bruit, PVD and/or CAD

Front 36

What should you look for in a young person (<50) who presents with a stroke? What tests should you order?

Back 36

1. vasculitis
2. hypercoagulable state
3. thrombophilia

Protein C and S, antiphospholipid antibodies, Factor V Leiden mutation, ANA, ESR, rheumatoid factor, VDRL/RPR, Lyme serology, TEE

Front 37

What should you order in any patient who presents to the ED with symptoms consistent with a stroke?

Back 37

1. Non-contrast head CT
2. ECG
3. CXR
4. coags- Platelets and CBC, PT and PTT
5. electrolytes
6. glucose level
7. bilateral carotid US
8. echocardiogram

Front 38

Huntington's Chorea - general characteristics
1. cause
2. age of onset and progression

Back 38

1. Autosomal dominant inheritance of a trinucleotide repeat disorder - CAG on chromosome 4. This leads to loss of GABA-producing neurons in the striatum
2. onset is between 30-50 years of age. Symptoms worsen steadily, with 15 years being typical duration from onset until death

Front 39

Clinical features of Huntington's chorea

Back 39

1. chorea- involuntary movements of the face, head and neck, tongue, trunk and extremities
2. Altered behavior- irritability, personality changes, antisocial behavior, depression, OCD, and/or psychosis
3. Impaired mentation - progressive dementia is a key feature- 90% of patients are demented before age 50
4. gait is unsteady and irregular. ultimately bradykinesia and rigidity prevail

Front 40

Diagnosis and treatment of Huntington's chorea

Back 40

1. diagnosis- MRI shows atrophy of the head of the caudate nuclei. DNA testing confirms the diagnosis. Genetic counseling is important since it is AD
2. Treatment is symptomatic- there is no cure. Dopamine blockers may help with the psychosis and improve the chorea. Anxiolytic and antidepressant therapy may also help

Front 41

Physiologic tremor - causes

Back 41

1. fear, anxiety, fatigue
2. metabolic- hypoglycemia, hyperthryoidism, pheochromocytoma
3. Toxic causes- alcohol withdrawal, valproic acid (depakote), lithium, methylxanthies-- caffiene and theophylline

Front 42

Essential tremor
1. cause
2. exacerbating factors
3. other features
4. treatment

Back 42

1. common condition that is inherited (autosomal dominant) in up to 1/3 of patients
2. induced or exacerbated by intentional activity, such as drinking from a cup or using utensils. Markedly decreased by alcohol intake, which may help in the diagnosis
3. distorted handwriting is often present. No bradykinesia, rigidity etc though
4. propanolol

Front 43

Features of ataxia

Back 43

1. gait instability
2. loss of balance
3. impaired limb coordination

Front 44

Causes of ataxia

Back 44

1. Acquired causes- alcohol intoxication, vitamin B12 or thiamine deficiency, cerebellar infarction or neoplasm, demyelinating disease (MS, AIDS and tertiary syphilis-tabes dorsalis)
2. Inherited causes-- Friedrich's ataxia - autosomal recessive inheritance, onset by young adulthood. Presents with ataxia, nystagmus, impaired vibratory sense and proprioception
- ataxia telangiectasia- AR inheritance, childhood onset, similar to Friedrich's ataxia except there are telangiectasias. Increased incidence of cancer

Front 45

Tourette's syndrome - general characteristics
1. associations
2. age of onset
3. cause
4. other notes

Back 45

1. assoc with OCD
2. onset before age 21
3. thought to have AD inheritance pattern
4. not all patients with tics have Tourette's and not all patients with Tourette's have coprolalia

Front 46

Clinical features of Tourette's syndrome and treatment. What must you rule out?

Back 46

1. motor tics (multiple)- facial grimacing, blinking, head jerking, shoulder shrugging, 2. phonic tics (at least one kind)- grunting, sniffing, throat clearing, coprolalia
2. clonidine- a2 agonist, pimozole, haloperidole
3. must r/o seizures, tardive dyskinesia and Huntington's

Front 47

Dementia
1. definition
2. most significant risk factor

Back 47

1. Dementia- progressive deterioration of intellectual function, typically characterized by preservation of consciousness
2. The most important risk factor is increasing age-- benign forgetfulness may also be a risk factor

Front 48

Differential diagnosis of dementia

Back 48

1. primary neurologic disorders (Alzheimer's disease- 66% of all cases, vascular dementia (multi-infarct, step-wise decline due to a series of cerebral infarctions, Binswager disease), space-occupying lesions such as brain tumor or chronic subdural hematoma, Normal pressure hydrocephalus- triad of dementia, incontinence and gait disturbance, dementia with lewy body, pick's dz, MS, parkinson's huntington's, wilson's
2. Infections - HIV, neurosyphilis, cryptococcal CSF infection, Creutzfeldt-Jakob dz, PML
3. metabolic disorders- thyroid dz, vitamin B12 def, thiamine def, niacin def
4. drugs or toxins- chronic alcoholism- may cause dementia independent of thiamine deficiency, aniline dyes, metals, lead
5. pseudodementia- depression

Front 49

Clinical approach to dementia

Back 49

1. patient history- ask patient and their family members about the nature of the onset of specific deficits, physical symptoms, comorbid conditions. review meds, social and fam hx
2. physical exam- thorough neuro exam and MSE. Focus on gait exam to look for evidence of movement disorders, mass lesions, and NPH
3. lab and imaging- CBC with diff, chem panel, thyroid function tests, vitamin B12, folate, VDRL, HIV, CT or MRI of head

Front 50

Treatment and management of dementia

Back 50

1. treat reversible causes
2. avoid and/or monitor doses of medications with adverse congitive side effects like glucocorticoids, opiates, sedative hypnotics, anxiolytics, anticholinergics, lithium
3. treat/control comorbid medical conditions - diabetes, HTN, depression visual/hearing impairment
4. pharmacologic therapy- vitamin E, tacrine, donepezil- evidence is inconclusive on these though

Front 51

Epidemiology of Alzheimer's disease
1. how common
2. prevalence with age

Back 51

1. 4th most common cause of death in the US
2. Prevalence increases with age- approx 10-15% of ppl over 65, 15-30% of ppl over 80. Most die of other causes first

Front 52

Risk factors for Alzheimer's disease

Back 52

1. Age
2. family history (especially if early-onset)
3. Down's syndrome

Front 53

Pathology (on autopsy) of Alzheimer's disease

Back 53

1. Quantity of senile plaques (age-specific) - focal collections of dilated, tortuous neuritic processes surrounding central amyloid core (amyloid beta-protein)
2. quantity of neurofibrillary tangles- bundles of neurofilaments in cytoplasm of neurons, denotes neuronal degeneration

Front 54

Clinical features of Alzheimer's
1. progression
2. onset to death
3. stages
4. advanced disease
5. cause of death

Back 54

1. begins insidiously but tends to progress steadily
2. the average time from onset to death is 5-10 years
3. stages: a. early- mild forgetfulness, impaired ability to learn new material, poor performance at work, poor concentration, changes in personality, impaired judgment (e.g. inappropriate humor), b. intermediate- memory is progressively impaired. patients may be aware of the condition, but denial is often present. Visiospatial disturbances are common-- getting lost in a familiar place and difficulty following directions, later stages-assistance needed for ADLs. Patients have difficult remembering the names of relatives/friends or major aspects of their lives. Paranoid delusions and hallucinations are common.
4. advanced - complete debilitation and dependence on others, incontinence, may even forget his/her own name
5. death is usually secondary to infection or other complications of a debilitated state

Front 55

Diagnosis of Alzheimer's disease

Back 55

1. essentially a clinical diagnosis- exclude other causes first
2. CT or MRI of brain shows diffuse cortical atrophy with secondary enlargement of the ventricles-- supports diagnosis

Front 56

Treatment of Alzheimer's disease

Back 56

1. cholinesterase inhibitors- brains of patients with Alzheimer's have lower level of Ach- avoid anticholinergics. Give donepezil, rivastigmine and/or galantamine. First-line
2. certain dietary supplements- gingko and lethicin-- not proven beneficial
3. Vitamin E- one study showed the mega doses (2000 IU/day) slowed progression and preserved function in people with moderately severe AD. Needs further study

Front 57

Dementia with Lewy Body

Back 57

1. has features of both Alzheimer's disease and Parkinson's, but progression may be more rapid than in Alzheimer's
2. initially, visual hallucinations predominate. Other symptoms include EPS and fluctuating mental status
3. these patients are often sensitive to the adverse effects of neuroleptic agents-- exacerbate symptoms
4. treatment is similar to alzheimer's diseases- but use neuroleptics for hallucinations and psychotic features. Selegiline (MAO-I) may slow dz progression

Front 58

Acute Confusional state/ Delirium
1. definition
2. whose is more prone to this

Back 58

1. Acute period of cognitive dysfunction due to a medical disturbance or condition- that is REVERSIBLE
2. Elderly patients are especially prone to delirium-- often when hospitalized

Front 59

Causes of Delirium

Back 59

P. DIMM WIT
P- post-operative state (compounded by pain meds)
D- dehydration and malnutrition
I- Infection (sepsis, meningitis, encephalitis, UTI etc)
M- medications and drug intoxications- TCAs, corticosteroids, anticholinergics, hallucinogens, cocaine
W- withdrawal states - from EtOH or benzos
I- inflammation, fever
T- trauma, burns
Also SMASHED - S- structural brain pathology, stroke, subdural or epidural hematoma, tumor, hydrocephalus, infection, M- Meningitis, mental illness, A- alcohol, acidosis, S- seizures- post-ictal, substrate def (e.g. thiamine), H- hypercapnia, hypoglycemia, hyperthermia, hyponatremia, hypoglycemia, hypoxia, hypotension, E- endocrine causes, D- drugs - barbs, benzos, CO, methanol, cyanide

Front 60

Clinical features of Delirium

Back 60

1. In contrast to both dementia and psychosis, delirium is characterized by a rapid deterioration of mental status (over hours to days), a fluctuating level of awareness, disorientation, and frequently abnormal vital signs
2. may be accompanied by acute abnormalities of perception, such as hallucinations
3. Patients may not necessarily be agitates, but may have a slow, blunted responsiveness

Front 61

Diagnosis of delirium

Back 61

1. Mental Status Exam- MMSE
2. Lab- chem panel, vitamin B12, thiamine
3. LP- perform in any febrile, delirious patient unless there are contraindications (e.g. cerebral edema, signs of inc ICP- to prevent brainstem herniation)

Front 62

Treatment of delirium

Back 62

1. treat the underlying cause
2. haloperidol- for agitation/psychotic-like delirious behavior
3. supportive treatment- frequent re-orienting etc

Front 63

Coma- general characteristics
1. definition
2. Causes

Back 63

1. depressed LOC to the extent that the patient is completely unresponsive to any stimuli
2. a. structural brain lesions that cause a coma are usually bilateral unless they produce enough mass effect to compress the brainstem or the opposite cerebral hemisphere
b. global brain dysfunction- metabolic or systemic disorders
c. psychiatric causes- conversion disorder or maliginering

Front 64

Approach to Coma Management
1. Initial Steps
2. Approach to diagnosis

Back 64

1. Assess vital signs. ABCs. Always assume there is an underlying trauma (stabilize C-spine) and assess for signs of underlying trauma. Assess level of consciousness with GCS. Keep repeating
2. Approach to diagnosis- Rapid motor exam- if asymmetry is noted in movements then a mass lesion is the likely cause. Metabolic or systemic causes do not produce these motor abnormalities
b. brainstem reflexes- pupillary light reflex, eye movement, breathing on own
c. lab tests- CBC, electrolytes, calcium, BUN, creatinine, glucose, plasma osmolarity, ABG, ECG
d. tox screen or blood and urine
e. CT or MRI of brain
f. LP - if meningitis or SAH is suspected

Front 65

Brainstem reflexes
1. if pupils are equal round and reactive (ERR)
2. asymmetric pupils
3. eye movement
4. breathing

Back 65

1. midbrain is intact and not the cause of the coma
2. anisocoria - may be a sign of uncal herniation. Leep in mind that certain eye drops of systemic meds may alter pupil size
3. eye movements- if the C-spine is uninjured perform the oculocephalic test "doll's eyes" to see if the patient's eyes track. When the head is turned to one side the eyes should move conjugately to the opposite direction if the brainstem is intact.
4. if the patient is breathing on his or her own then the brainstem is functioning

Front 66

Treatment of coma

Back 66

1. Correct reversible causes- control airway, give supplemental O2, naloxone for narcotic OD, dextrose (for hypoglycemia). Give thiamine before a glucose load. Correct and abnormalities in BP, electrolytes or body temperature
2. Identify and treat herniation- lowering the ICP is critical

Front 67

"Locked In Syndrome"
- what is it?
- what usually causes it?

Back 67

- mimics coma, because the patient is completely paralyzed with sparing of the muscles required for respiration, blinking and vertical eye movement
- patients are FULLY AWARE of their surrounding and capable of feeling pain
- usually caused be infarction or hemorrhage of the ventral pons

Front 68

Multiple Sclerosis- pathology

Back 68

1. selective demyelination of the CNS- multifocal zones of demyelination (plaques) are scattered throughout the white matter. Classic locations of hte plaques is at the angles of the lateral ventricles
- demyelination primarily involves the white matter of the brain and spinal cord-- tends to spare gray matter/axons and the peripheral nervous system. However, there may be more cortical demyelination than previously thought
- commonly involved tracts: pyramidal and cerebellar pathways, medial longitudinal fasciculus, optic nerve, posterior columns

Front 69

Who is more likely affected by MS and what is the etiology of the disease?

Back 69

Women are 2-3 times more likely to be affected than men
- the etiology is unknown, but is probably secondary to interplay of environmental, immunologic and genetic factors

Front 70

Clinical features of MS

Back 70

1. Transient sensory deficitis - most common initial presentation, and decreased sensation or paresthesias in upper and lower limbs
2. fatigue- common complaint
3. motor symptoms- mainly weakness or spasticity- may appear insidiously or acutely. Caused by pyramidal tract involvement-- UMN involvement. Spasticity (such as leg stiffness) can impair the patient's ability to walk and maintain balance. Can lead to weakness and progression to paraparesis, hemiparesis or quadriparesis
4. visual disturbances - optic neuritis- monocular vision loss (in up to 20% of patients), pain on eye movement, central scotoma, decreased pupillary reaction to light, Intranuclear ophthalmoplegia - INO- strongly suggests the diagnosis- ipsilateral medial rectus palsy on attempted lateral gaze and horizontal nystagmus of the abducting eye (contralateral to the side of the lesion)
5. cerebellar involvement- ataxia, intention tremor, dysarthria
6. loss of bladder control- UMN injury in spinal cord
7. autonomic involvement- impotence and/or constipation
8. cerebral involvement- may occur in advanced disease- memory loss, personality change, emotional lability, anxiety and depression
9. neuropathic pain- hyperesthesias and trigeminal neuralgia- frustrating and common

Front 71

Intranuclear ophthalmoplegia (INO)

Back 71

strongly suggests the diagnosis of MS - ipsilateral medial rectus palsy on attempted lateral gaze and horizontal nystagmus of the abducting eye (contralateral to the side of the lesion) this is due to a lesion of the medial longitudinal fasiculus
- diplopia can occur

* picture show a Right INO/MLF lesion - patient is attempting to look left. Left eye has nystagmus, but right eye has a medial rectus palsy and does not move at all

Front 72

Course of Multiple Sclerosis
1. age of presentation
2. variants of progression

Back 72

1. most patients are in their 20s to 30s at the time of presentation and have a localizing deficit such as optic neuritis, one-sided weakness, or numbness. May or may not develop MS from there
2. clinically silent- stable or benign MS- some progression may occur later in the course of the disease
- relapsing/remitting- most common- exacerbations followed by remissions.
- secondary progressive - patients with relapsing/remitting disease can experience a worsening of their symptoms that is progressive in later years
- primary progressive- this is a steady progressive disease that appears later in life (after 40 yo) and tends to have less visual and more axonal involvement

Front 73

How often do attacks of MS usually occur and what is the prognosis? What signs imply a worse prognosis?

Back 73

1. Attacks average about once per year. No one precipitant has been proven to cause them
2. Prognosis is highly variable, with a normal life span in most patients
- although the quality of life is diminished, many patients never develop debilitating disease
- approx 1/3 of patients eventually progress to severe disability-
- prognostic factors that increase the likelihood of severe disability- frequent attacks early in the disease course, onset at a later age, progressive course, and early cerebellar or pyramidal involvement

Front 74

Diagnosis of MS

Back 74

1. The diagnosis is essentially clinical- suspect it in young adults with relapsing and remitting signs and symptoms that difficult to explain (due to involvement of different areas of the CNS white matter). Nevertheless, once suspected, order an MRI and consider an LP
2. MRI is the test of choice (most sensitive) in the diagnosis - now considered standard of care, sensitive in identifying demyelinating lesions in the CNS, the number of lesions on the MRI is NOT necessarily proportional to disease severity or speed of progression
3. LP and CSF analysis- no lab tests are specific- but oligoclonal bands or IgG are present in 90% of MS patients
4. Evoked potentials can suggest demyelination of certain areas by measuring the speed of nerve conduction within the brain. Newly remyelinated nerves conduct sensory impulses more slowly

Front 75

Treatment of acute MS attacks

Back 75

1. High dose IV corticosteroids can shorten an acute attack. Oral steroids have not shown the same efficacy
- studies have shown that treatment of acute exacerbations does NOT alter the outcome or course of MS
- most acute attacks resolve within 6 weeks with or without treatment
2. disease modifying treatment
- interferon therapy. Recombinant interfern B-1a, B-1b and glatiramer decrease relapse rates mildly
- interferons cause flu-like symptoms, which can be severe and persistent.
- this should be started early in the course of the disease before the disability becomes irreversible. Long term benefits are unknown
- non-specific immunosuppressive therapy such as cyclophosphamide should be reserved for rapidly progressive disease, because of the toxic side effects
4. symptomatic therapy- baclofen or dantrolene for muscle spasticity. carbamezapine or gabapentin for neuropathic pain. Treat depression as indicated

Front 76

Guillain-Barre Syndrome- general characteristics
1. what is it?
2. what causes it?
3. associations?

Back 76

1. inflammatory demyelinating polyneuropathy that primarily affects the motor nerves
2. usually preceded by a viral or mycoplasmal infection of upper respiratory or GI tract. Common infections include campylobacter jejuni, CMV, hepatitis and HIV
3. may also occur in Hodgkin's disease, lupus, after surgery or after HIV seroconversion

Front 77

Clinical features of Guillain Barre Syndrome

Back 77

1. abrupt onset with rapidly ascending weakness/paralysis of all four extremities which frequently progresses to involve respiratory, facial, and bulbar muscles - usually symmetric (not always), weakness may be mild or severe, weakness usually progresses from distal to central muscles. If generalized paralysis is present-- it can lead to respiratory arrest
2. extremities may be painful, but sensory loss is NOT typical
3. Sphincter control and mentation are typically spared
4. autonomic features (arrhythmias, tachycardia, postural hypotension) are dangerous complications

Front 78

Diagnosis of Guillain Barre syndrome

Back 78

1. CSF analysis- elevated protein, but normal cell count
2. electrodiagnostic studies- decreased motor nerve conduction velocity
3. supporting clinical findings/history

Front 79

Treatment of Guillain Barre syndrome

Back 79

1. carefully monitor pulmonary function. mechanical ventilation may be necessary
2. administer IV immunoglobulin if the patient has significant weakness. If progression continues, plasmapharesis may reduce the severity of the disease
3. Do NOT give steroids- they are usually harmful and never helpful

Front 80

Prognosis for Guillain Barre syndrome

Back 80

- signs of recovery within 1-3 weeks after onset favors a good prognosis. If illness continues for a longer period (e.g. beyond 6 weeks) a chronic relapsing course is more likely and the prognosis is less favorable
- it may take months before the patient recovers. A minority of patients experience recurrent attacks and about 5% die due to respiratory failure, pneumonia, or arrhythmias

Front 81

Myasthenia Gravis
1. etiology
2. epidemiology

Back 81

1. Autoimmune disorder- autoantibodies are directed against NAD receptors in the neuromuscular junction, which leads to reduced post-synaptic response to ACh and results in significant muscle fatigue
2. muscles that are stimulated repeatedly (e.g. extraocular muscles) are prone to fatigue
3. The peak incidence in women is age 20-30. In men it is 50-70 (bimodal distribution). It is more common in women

Front 82

Clinical features of myasthenia gravis
1. key features
2. what muscle groups are affected
3. most common initial presentation

Back 82

1. skeletal muscle weakness- with preservation of sensation and reflexes. Weakness is exacerbated by continued use of the muscle and improved by rest. Symptoms worsen toward the end of the day due to fatigue
2. Involved muscles vary and may include: EOM, eyelids (ptosis), facial muscles (facial weakness, difficulty chewing, slurred speech), limb muscles (proximal and asymmetric).
3. Ptosis and blurred vision are the most common initial symptoms
4. generalized weakness, dysarthria, and dysphagia
5. The condition progresses slowly with periodic exacerbations. Myasthenic crisis is a medical emergency that occurs in 15% of patients. Diaphragm and intercostal fatigue result in respiratory failure, often requiring mechanical ventilation

Front 83

Lambert-Eaton Syndrome
1. features
2. associations
3. causes/pathogenesis
4. how to differentiate from similar syndromes

Back 83

1. proximal muscle weakness and hyporeflexia
2. associated with small cell lung cancer
3. caused by autoantibodies directed against pre-synaptic calcium channels
4. distinguished from myasthenia gravis in that symptoms IMPROVE with repeated muscle stimulation

Front 84

Diagnosis of Myasthenia Gravis

Back 84

1. Ach receptor antibody test is the test of choice. Nevertheless, 20% of patients with clinical manifestations may be "antibody negative"
2. EMG shows decremental response to repetitive stimulation of motor nerves
3. A CT scan of the thorax can rule out thymoma. Thymoma is present in only 10-15% of patients, but the thymus is histiologically abnmormal in 75%
4. Edrophonium (Tensilon) test- Acetylcholinesterase (ACHe) medications cause marked improvement of symptoms, but a high false-positive rate limits the utility

Front 85

Treatment of myasthenia gravis
1. first-line treatment
2. role of surgery
3. second and third line agents
4. options for myasthenic crisis/resp failure
5. new alternative treatments
6. PFTs

Back 85

1. AChE inhibitors- e.g. pyridostigmine. Inhibits the breakdown of ACh so that there is more in the synapse to stimulate the post-synaptic receptor. Symptomatic benefit only
2. Thymectomy- provides a symptomatic benefit and complete remission in may patients even in the absence of a thymoma. Although usually benign, thymoma is absolute indication for thymectomy
3. Immunosuppressive drugs - use corticosteriods for patients with a poor response to AChE inhibitors. Azathiprine and cyclosporine are alternative third line
4. Plasmapheresis removes antibodies to ACh receptor. Use if all else fails of the if the patient is in respiratory failure
5. IV immunoglobulin therapy is now sometimes used for acute exacerbations
6. Monitor serial forced VCs. A forced VC of 15 mL/kg ( about 1L) is generally an indication for intubation. Patients with myasthenic crisis have a low threshold for intubation-- do not wait for hypoxia to set in

Front 86

Which medications may potentially exacerbate Myasthenia Gravis symptoms?

Back 86

1. antibiotics- aminglycosides and tetracyclines
2. beta-blockers
3. antiarrhythmics- quinidine, procainamide and lidocaine

Front 87

Duchenne's Muscular Dystrophy
1. cause
2. is there inflammation present?

Back 87

1. X-linked recessive (almost exclusively in males) disease that involves a mutation on the gene that codes for the dystrophin protein (dystrophin is absent causing muscle cells to die)
2. Characteristically, there is NO inflammation

Front 88

Clinical features of Duchenne's muscular dystrophy

Back 88

1. muscle weakness that is progressive, symmetric and starts in childhood. Proximal muscles primarily affected (pelvic girdle). Eventually involves the respiratory muscles
2. Gower's maneuver- patient uses hands to get up from the floor because the weakness in the proximal lower extremities makes it difficult to rise without support
3. enlarged calf muscles- true muscle hypertrophy at first, followed by pseudohypertrophy as fat replaces muscles
4. ultimately results in wheelchair confinement, respiratory failure and death in the third decade

Front 89

Diagnosis of Duchenne's muscular dystrophy

Back 89

1. serum creatine phosphokinase (CPK)- levels are markedly elevated signifying muscle degradation
2. DNA testing has now replaced muscle bx for dx

Front 90

Treatment of Duchenne's muscular dystrophy

Back 90

1. Prednisone is beneficial and is associated with a significant increase in strength, muscle function, and pulmonary function. It may reduce the risk of scoliosis. Chronic steroid treatment does have side effects, but it is recommended for boys 5 yo and older whose motor skills are declining
2. Surgery to correct progressive scoliosis is often necessary once the patient becomes wheelchair dependent

Front 91

Becker's Muscular Dystrophy
1. prevalence
2. inheritance pattern
3. clinical features

Back 91

1. Less common that Duchenne's muscular dystrophy
2. also X-linked recessive
3. similar to Duchenne's muscular dystrophy, but there is a later onset and less severe course. Some dystrophin is present

Front 92

Hereditary causes of muscles weakness

Back 92

1. Duchenne's and Becker's muscular dystrophies
2. mitochondrial disorders associated with maternal inheritance and ragged red muscle fibers
3. Glycogen storage diseases such as McArdle's disease (autosomal recessive, muscle cramping after exercise due glycogen phosphorylase deficiency).

Front 93

Neurofibromatosis Type I - NF1 (von Recklinghausen's disease)
1. cause/inheritance pattern
2. clinical features
3. complications
4. treatment

Back 93

1. autosomal dominant disease
2. characterized by cafe au lait spots, neurofibromas, CNS tumors (gliomas, meningiomas), axillary or inguinal freckling, iris hamartomas (Lisch nodules), bony lesions. Cutaneous neurofibromas which may be disfiguring
3. Complications include scoliosis, pheochromoctyomas, optic nerve gliomas, renal artery stenosis, and erosive bone defects. MSK manifestations include spinal deformity and congenital tibial dysplasia
4. complications may require treatment. Surgically excise and symptomatic neurofibromas

Front 94

Neurofibromatosis Type II (NF2)

Back 94

Autosomal dominant disease that is less common than NF1
- clinical features include bilateral (sometimes unilateral) acoustic neuromas (classic finding), multiple meningiomas, cafe au lait spots, neurofibromas (much less common than type I), cataracts

Front 95

Tuberous sclerosis
1. inheritance pattern
2. presentation
3. other features
4. treatment

Back 95

1. usually AD
2. presents with cognitive impairment, epilepsy, and skin lesions (including facial angiofibromas, adenoma sebaceum)
3. retinal hamartomas, renal angiomyolipomas, and rhabdomyomas of the heart may also be present
4. treat complications

Front 96

Sturge-Weber syndrome

Back 96

1. acquired disease
2. key pathologic feature is the presence of capillary angiomatoses of the pia mater
3. major clinical feature is a facial vascular nevi (port wine stain)
4. epilepsy and MR are usually present
5. treatment of epilepsy is often the mainstay of treatment

Front 97

Diagnosis?

Back 97

Sturge-Weber syndrome

Front 98

What is the prognosis of neurofibromatosis?

Back 98

It depends on the type and number and location of the tumors. Most patients can function well.

Front 99

Von Hippel Lindau Disease
1. inheritance pattern
2. clinical features
3. associations

Back 99

1. Autosomal dominant
2. cavernous hemangiomas of the brain or brainstem, renal angiomas, and cysts in multiple organs
3. associated with renal cell carcinoma
4. associated with pheochromocytomas

Front 100

What is this and what is a possible diagnosis?

Back 100

Cavernous hemagioma-- possible dx = Von Hipple Lindau (VHL) syndrome

Front 101

Syringomyelia

Back 101

1. central cavitation of the cervical cord to abnormal collection of fluid within the spinal cord parenchyma
2. most commonly associated with Arnold-Chiari malformation. Other causes are post-traumatic, postinfectious, tethered cord and intramedullary tumors
3. clinical features- most often asx and discovered incidentally on MRI obtained for other reasons. Sx may include b/l loss of pain and temperature sensation over the shoulders in a capelike distribution (lateral spinothalamic tract involvement), with preservation of touch, thoracic scoliosis and muscle atrophy of the hands may occur
4. diagnosed by MRI
5. Treatment depends on the size of syrinx, symptoms and associated findings (chiari, tethered cord etc). Eval by neurosurg recommended.

Front 102

Diagnosis?

Back 102

Arnold Chiaria malformation

Front 103

Brown-Sequard syndrome
1. what is it
2. causes - which are most common?
3. clinical features
4. prognosis

Back 103

1. spinal cord hemisection (i.e. lesions involving either the right or left half of the spinal cord (usually at teh cervical levels (where spinal cord enlarges)
2. causes include trauma (fracture or stab wound), that causes hemisection of the spinal cord most commonly, a crush injury to one side of the spinal cord, tumors and abscesses less commonly
3. clinical features- contralateral loss of pain and temperature (spinothalamic tract) and ipsilateral hemiparesis (corticospinal tract) and ipsilateral loss of position/vibration sense (dorsal columns)
4. prognosis for neurologic recovery is good
- difference in sides affected (ipsilateral vs contralateral) is due to where tracts decussate- ST tract crosses at the level of the injury

Front 104

Transverse myelitis
1. what is it?
2. cause
3. clinical features
4. diagnosis
5. treatment
6. prognosis

Back 104

1. this is a rare condition that specifically affects the tracts across the horizontal aspect of the spinal cord at a given level. The thoracic spine is the most commonly involved
2. The causes is unknown, but it can occur after viral infections. Progression is usually rapid
3. clinical features include lower extremity weakness or plegia, back pain, sensory deficits below the level of the lesion, and sphincter disturbance (esp urinary retention)
4. Diagnosis - MRI with contrast
5. tx- steroid therapy is often used, but evidence supporting its use is equivocal.
6. prognosis is highly variable and unpredictable, ranging from full recovery to death

Front 105

Horner's syndrome - general characteristics
1. cause
2. location of lesion

Back 105

1. results from the interruption of cervical sympathetic nerves
2. can be preganglionic (central lesions) or postganglionic (distal to superior cervical ganglion). The former is more worrisome and requires more thorough evaluation

Front 106

Clinical features of Horner's syndrome

Back 106

1. Ipsilateral pstosis - mild drooping of the lid "levator palpeprae still intact"
2. ipsilateral miosis - "pin point pupil"
3. ipsilateral anhidrosis- decreased sweating on the forehead - may be difficult to assess

Front 107

Causes of Horner's syndrome

Back 107

1. Idiopathic (most cases)
2. Pancoast tumor (pulmonary neoplasm of the superior sulcus at lung apex)
3. Internal carotid dissection
4. brainstem stroke
5. neck trauma

Front 108

Poliomyelitis
1. what does it affect
2. characteristic features
3. treatment

Back 108

1. Poliovirus affects the anterior horn cells and motor neurons of the spinal cord and brainstem. Causes LMN involvement
2. Characteristic features include asymmetric muscle weakness (legs more commonly involved), absent DTRs, flaccid, atrophic muscles and normal sensation
3. Bulbar involvement (of CN IX and CNX) in 10-15% of cases can lead to respiratory and cardiac impairment
4. No treatment available, although it is mostly eradicated- prevented by vaccination

Front 109

Dizziness
1. three major causes/types
2. conditions that can cause the sensation of dizziness

Back 109

1. Presyncope (lightheadedness), Vertigo, multisensory stimuli this happens in times of profound shock or overwhelming sensory overload
2. may conditions can causes this -- cerebellar disease, cerebrovascular disease, TIAs, hyperventilation, anxiety, panic attacks and phobias

Front 110

Diagnosis of dizziness-- too differentiate cause

Back 110

1. Audiogram-- if vestibular symptoms present
2. CT scan/MRA- if TIA suspected
3. MRI of the posterior fossa if structural lesion suspected

Front 111

Vertigo
1. what is it
2. goal of diagnostic approach
3. peripheral vs central causes/implication

Back 111

1. disturbance of the vestibular system characterized by sensation of spinning or hallucination of movement
2. The initial goal is to determine whether the causes of the vertigo is peripheral (inner ear) or central (e.g. tumor, CVA)
3. Peripheral vertigo is usually benign, but central vertigo can have serious consequences

Front 112

Types/causes of peripheral vertigo (5)

Back 112

1. Benign Positional Vertigo (BPV)
2. Meniere's disease
3. acute labyrinthitis
4. ototoxic drugs
5. acoustic neuroma (schwannoma) - 8th cranial nerve (vestibulocochlear) - ataxia, gait unsteadiness, nystagmus, hearing loss, tinnitus

Front 113

Benign Positional Vertigo (BPV)
1. what is it
2. who does it usually occur in
3. treatment and prognosis

Back 113

- vertigo is experiences only in specific positions or during change of position that lasts for a few moments. It has an abrupt onset as soon as the particular position is assumed.
- usually presents in patients over 60 yo.
- treat with meclazine. Also teach patient otolith repositioning maneuvers
- recovery is usually complete (resolves within 6 mo)

Front 114

Meniere's disease

Back 114

1. Triad of vertigo, tinnitus, and hearing loss
2. attacks may last for hours to days and recur several months or years later
3. the hearing loss eventually becomes permanent
4. treat with sodium restriction and diuretics

Front 115

Acute labyrinthitis
- what causes it

Back 115

1. due to viral infection of the cochlea and labyrinth, may last for several days

Front 116

Ototoxic drugs

Back 116

amingoglycosides, some diuretics

Front 117

Causes of central vertigo

Back 117

1. MS- demyelination of the vestibular pathways of the brainstem
2. verterbrobasilar insufficiency
3. migraine-associated vertigo- HA may or may not be present

Front 118

Syncope - general characteristics
1. definition and recovery

Back 118

1. transient loss of consciousness/postural tone secondary to acute decrease in cerebral blood flow
2. It is characterized by rapid recovery of consciousness without resuscitation

Front 119

Causes of syncope

Back 119

1. seizure disorder
2. cardiac
3. vasovagal- neurocardiogenic- vasodepressor- simple faint
4. orthostatic hypotension
5. severe cerebrovascular disease
6. metabolic causes - hypoglycemia, hyperventilation, hypovolemia, hypersensitivity to carotid sinus pressure, mechanical reduction of venous return (valsalva or post-micturition), various meds (b-blockers, nitrates, antiarrhythmics)

Front 120

Cardiac syncope
1. clinical features
2. causes

Back 120

1. usually sudden and without prodomal symptoms- e.g. patient's face hits the floor. This may be the first manifestation of a life-threatening cardiac condition.
2. causes include: arrhythmias- sick sinus syndrome, vtach, AV block, rapid SVT. Obstruction to blood flow- aortic stenosis, hypertrophic cardiomyopathy, pulmonary HTN, atrial myxoma, prolapsed MV, severe assymetric septal hypertrophy (HOCM). Massive MI

Front 121

Vasovagal syncope - neurocardiogenic, vasodepressor, simple faints
1. how common
2. course of "illness"
3. clues to the diagnosis
4. treatment

Back 121

1. most common cause of syncope- may account for 50% of all cases of syncope
2. most people have one episode, but for some it is a recurrent problem
3. clues to diagnosis- emotional stress, fear, pain, extreme fatigue or claustrophobia. Prodomal symptoms- pallor, diaphoresis, lightheadedness, nausea, dimming of vision, roaring in the ears. Can occur at any age, but if the first episode is after 40, be reluctant to make the dx. Tilt table study can reproduce symptoms in susceptible patients
4. can usually be reversed by assuming the supine position and elevation of the legs to increase venous return to the heart. B-blockers and disopyramide may be helpful. Prognosis is excellent

Front 122

Pathophysiology of vasovagal syncope/syndrome

Back 122

- normally standing up causes the blood to pool in the LEs (leading to a decrease in CO, SV and BP). These changes are compensated for by an increased sympathetic tone (leading to vasoconstriction and tachycardia) and decreased parasympathetic tone.
- in patients with vasovagal syndrome, the compensatory response is interrupted in a few minutes by a paradoxical withdrawal of sympathetic stimulation and a replacement by enhanced parasympathetic (vagal) activity. This leads to inappropriate bradycardia, vasodilation, marked decrease in BP and cerebral perfusion

Front 123

Orthostatic hypotension
1. cause
2. who does it usually present in?
3. clinical features/clues to diagnosis
4. treatment

Back 123

1. defect in the vasomotor reflexes, overlaps with vasovagal syncope (ganglionic blocking agents, diabetes, old age, prolonged bed rest)
2. common in elderly people, diabetics (autonomia neuropathy), patients taking ganglionic blocking agents, vasodilators, diuretics
3. posture is the main cause here - sudden or prolonged standing are the precipitating causes. A positive tilt-table test result is expected. It is also associated with premintory symptoms (LH, nausea etc)
4. Treat with increased sodium intake and fluids. Consider fludrocortisone (synthetic aldosterone)

Front 124

"Drop attacks" / cerebrobasilar insufficiency

Back 124

1. a rare cause of syncope
2. A TIA involving the vertebrobasilar circulation can lead to syncope- drop attacks.
3. one practically never experiences dizziness or vertigo in isolation with this-- there will always be other deficits as well

Front 125

Diagnostic approach to syncope

Back 125

1. First, attempt to r/o conditions that are life threatening (e.g. MI, hemorrhage, and arrhythmias)
2. The main goal is to differentiate between cardiac and non-cardiac etiologies because the prognosis is the poorest for those with underlying heart disease
3. History- 3 key elements need to be determined-- events before, during and after the syncopal episode. Check the patient's medications (esp in elderly). See reports from witnesses.
4. Physical exam (priority given to CV system) - BP and pulse measurements in supine, sitting, and standing positions, mental status exam (post-ictal?), murmurs (aortic stenosis, HCM), carotid pulses - auscultate for bruits, apply pressure to carotid sinus and watch for reflex bradycardia and hypotension
5. ECG can identify life-threatening causes (VTach, other arrhythmias, ischemia). Obtain in ALL patients
6. CBC, metabolic panel
7. additional diagnostic tests- holter, tilt--table, CT or EEG, Echo, electrophys studies in select pts

Front 126

Seizures
- what are they
- seizures vs epilepsy

Back 126

- A seizure occurs when there is a sudden abnormal discharge of electrical activity in the brain
- the diagnosis of epilepsy is reserved for the syndrome or recurrent, idiopathic seizures. The ultimate cause of seizures in epilepsy is unknown.

Front 127

Causes of seizures

Back 127

Four Ms , Four Is
1. Metabolic and electrolyte disturbances- hyponatremia, water intoxication, hypoglycemia or hyperglycemia, hypocalcemia, uremia, thyroid storm, hyperthermia
2. Mass lesions- brain mets, primary brain tumors, hemorrhage
3. Missing drugs- noncompliance with anticonvulsants in patients with epilepsy. Acute withdrawal from alcohol, benzos, barbs
4. Miscellaneous - pseudoseizures- no true seizures but are psychiatric in origin, Eclampsia- only definitive treatment is deliver but magnesium should be given. Hypertensive encephalopathy
5. Intoxications- cocaine, lithium, lidocaine, theophylline, metal poisoning, CO
6. Infections- septic shock, bacterial or viral meningitis, brain abscesses
7. Ischemia- TIA, stroke- common in elderly
8. Increased ICP- due to trauma etc

Front 128

Partial seizure vs generalized seizure

Back 128

Partial seizures account for 70% of patients with epilepsy who are >18. It begins in one part of the brain (typically in the temporal lobe) and initially produces symptoms that are referable to the region of the cortex involved.
- Generalized seizures are characterized by LOSS of CONSCIOUSNESS- involved the disruption of electrical activity in the entire brain

Front 129

Types of partial seizures

Back 129

1. simple partial seizure- consciousness remains intact. The seizure remains localized but may evolve into a complex partial seizure. May involve transient unilateral tonic clonic movement
2. complex partial seizure- consciousness is IMPAIRED but not lost- post ictal confusion is present. There are automatisms (last 1-3 minutes)- purposeless, involuntary, repetitive movements (such as lip smacking or chewing), patients may become aggressive is restraint is attempted. Olfactory or gustatory hallucinations are common (e.g. smell of burning rubber- common prodomal sx)

Front 130

Tonic-clonic (grand mal seizure)

Back 130

1. Begins with a sudden loss of consciousness- a fall to the ground
2. tonic-phase-- the patient becomes rigid, trunk and limb extension occurs. The patient may become apneic during this stage
3. clonic stage- there is musculature jerking of the limbs and body for at least 30 seconds
4. The patient then becomes flaccid and comatose before regaining consciousness
5. Other features may include: tongue biting, vomiting, apnea, and incontinence (urine or feces)

Front 131

Absence (petit mal seizure)
- features
- typical presentation
- how frequently do they occur
- consciousness impaired or preserved?
- EEG pattern
- treatment

Back 131

1. Typically involves school-age children- and usually revolves as child grows older
2. patient seems to disengage from current activity "stare into space" then returns to activity several seconds later- patient looks absent minded and this is often confused with day dreaming of ADHD
3. episodes are brief - lasting a few seconds but they may be frequent- up to 100 times a day
4. impairment of consciousness but no loss of postural tone or continence and no post ictal confusion. Minor clonic activity (eye blinks or head nodding) is seen in 45% of patients
-- characteristic 3hz spike and wave pattern on EEG
- treat with Ethosuxamide

Front 132

Diagnostic approach to seizures

Back 132

1. if the patient has a known seizure disorder than check anticonvulsant levels- this is usually the only test needed. Because therapeutic anticonvulstant levels are variable, one dose may be toxic for one patient and therapeutic for another
2. If patient's history is unclear or if it is the patient's first seizure: get CBC, electrolytes, blood glucose, LFTs, renal function tests, serum calcium, UA. EEG - although abn EEG is no diagnostic. A normal EEG in a patient with a first seizure confers a better prognosis. CT scan of the head or MRI of the brain with and without gad.
- LP and blood cultures if patient is febrile (? meningitis)

Front 133

Treatment of seizures- general principles

Back 133

1. For all seizures, ABCs first- secure airway and roll patient on their side to prevent aspiration
2. patients with a history of epilepsy- these are usually due to non-compliance with medications- even one missed dose can be problematic. Give a loading dose of the anticonvulsant and then continue their regular regimen as before. These patients should be a chronically managed by a neurologist. Treatment with one of the standard antiepileptic drugs provides adequate control in 70% of adults. Some need more than one
- If seizures persist, increase the dosage of the first anticonvulsant until signs of toxicity appear. If still not controlled then add a second anticonvulsant.
- If seizures are controlled, have the patient continue medication for at least 2 years. If patient remains seizure free then may try taper. Confirm lack of seizure activity with EEG

Front 134

Treatment of patients with first seizure

Back 134

1. EEG and neuro consult
2. Anticonvulsant therapy -weight risks vs benefits of treatment and the risk of recurrence before initiating
3. With a normal EEG, risk of recurrence is relatively low - 15% in 1 yr, compared to 41% if EEG is abn
4. Do not treat patients with a single seizyre. Antiepileptic drugs are started if EEG is abn, brain MRI is abn or patient is in status epilepticus

Front 135

Anticonvulsant Agents
1. for generalized tonic clonic seizures and partial seizures
2. for petit mal (absence seizures)

Back 135

1. phenytoin and carbamezapine are the drugs of choice. They are equally effective and side-effect profiles are similar. Other options include phenobarbital, valproate, and primidone
2. For absence seizures - ethosuxamide and valproic acid are preferred

** These drugs are teratogens-- so always do a pregnancy test before prescribing**

Front 136

Amytrophic Lateral Sclerosis (ALS)- Lou Gehrig's disease - general characteristics

Back 136

1. a disorder affecting the anterior horns cells and the corticospinal tracts at many levels. Corticobulbar involvement is common as well. The presence of UMN and LMN signs is a hallmark of ALS. Note that ONLY the motor system is involved
2. Onset is usually between 50-70 yo. Occurrence before age 40 is rare
3. Only 10% of the cases are familial and the rest are sporadic of unknown etiology
4. Prognosis is dismal: 80% mortality rate at 5 years and 100% mortality rate at 10 years

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Clinical features of ALS

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1. progressive muscle weakness is the hallmark feature. Usually first noted in legs or arms, but then spreads to other muscle groups. No associated pain. There is muscle atrophy
2. Muscle cramps and spasticity
3. fasiculations unnoticed by patient (UMN sign)
4. impaired speech and swallowing. Dysphagia can lead to aspiration
5. respiratory muscle weakness. Dysnpean on exertion, later at rest, orthopnea, sleep apnea, end-stage ALS is characterized by respiratory failure
6. Weight loss and fatigue
7. The following are UNAFFECTED: bowel and bladder control, sensation, cognitive function, EOM, sexual function

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Diagnosis of ALS

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1. There is no specific diagnostic test for ALS. EMG and nerve conduction studies can confirm degeneration of the LMN and can r/o neuromuscular junction disorders
2. Clinical or electrical evidence. Involvement of two regions (probable ALS). Involvement of three or four regions (definite ALS)- affected regions include the bulbar (face, larynx, tongue, jaw), cervical and thoracic and lumbrosacral

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Treatment of ALS

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1. mainly supportive
2. riluzole is a glutamate-blocking agent- it may delay death by only 3-5 months

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Aphasia- general characteristics

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1. loss or defect of lanuague (e.g. speaking, fluency, reading, writing, comprehension or written or spoken material)
2. Most lesions that cause aphasia involve the dominant hemisphere, which is the left hemisphere in 95% of right handers and 50% of left handers

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Four types of aphasia

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1. Wernicke's aphasia
2. Broca's aphasia
3. conduction aphasia
4. global aphasia

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Wernicke's aphasia

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- RECEPTIVE fluent aphasia
- impaired comprehension of written or spoken language
- speech is gramatically correct and is fluid but does not make much sense
- patients articulate well but often use the wrong words because they cannot understand their own words
- "word salad"

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Broca's aphasia

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- EXPRESSIVE, nonfluent aphasia
- speech is slow and labored
- the patient uses short sentences (as few words as possible) without grammatical construction. The content is appropriate and meaningful.
- good comprehension of language (written and spoken)
- often associated with right hemiparesis and hemisensory loss

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Conduction aphasia

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- disturbance in REPETITION -- involves the connections between Broca's and Wernicke's areas (arcuate fasiculus)

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Global aphasia

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- disturbance in all areas of language function (comprehension, speaking, reading, fluency)
- often associated with right hemiparesis

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Causes of aphasia

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1. stroke (by far the most common cause)
2. trauma to the brain
3. brain tumor
4. Alzheimer's disease

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Treatment of aphasia

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1. most patients spontaneously recover or improve within the first month
2 speech therapy is helpful, but is unlikely to have much benefit after the first few months

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Bell's Palsy - general characteristics
- features
- prognosis

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- hemifacial weakness/paralysis of muscles innervated by CNVII due to swelling of the cranial nerve
- prognosis is very good- 80% recover fully within weeks to months

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Causes of Bell's palsy

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1. idiopathic
2. possible viral etiology- herpes simplex - immunologic and ischemic factors implicated as well
3. URI is a common preceding event
4. Lyme disease (borrelia burgdoferi)

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Clinical features of Bell's palsy

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- acute onset of unilateral facial weakness/paralysis. Both upper and lower parts of the face are affected

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Diagnosis of Bell's palsy

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1. clinical diagnosis, but consider Lyme disease in endemic regions as the approach to treatment is different
2. do NOT use steroids if Lyme disease is suspected
3. Consider EMG testing if paresis fails to resolve within 10 days

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Treatment of Bell's palsy

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1. usually non-required, as most cases resolve in 1 month
2. short course of steroid therapy (prednisone) and acyclovir
3. patient should wear eye patch at night to prevent corneal abrasion (cornea is exposed due to weakness of orbicularis oculi muscle that closes the eyelid)
4. surgical decompression of CNVII is indicated if the paralysis progresses or if tests reveal further deterioration

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Trigeminal Neuralgia- general characteristics and clinical features

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1. one of the most painful conditions known to mankind. usually idiopathic in origin
2. brief (second to minutes) but frequent attacks of severe, lancinating facial pain. It involves the jaw, lips, gums, and maxillary area (ophthalmic division is less commonly affected). Recurrent attacks may continue for weeks at a time. No motor or sensory paralysis

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Diagnosis of Trigeminal Neuralgia

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1. clinical diagnosis
2. MRI to rule out cerebellopontine angle (CPA) tumor

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Treatment of Trigeminal Neuralgia
1. drug of choice
2. if medical tx fails...
3. course

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1. carbamezapine- usually effective in relieving pain. Other choices are baclofen and phenytoin, either alone or in combination with carbamezapine
2. consider surgical decompression if medical therapy fails
3. patients typically experience a relapsing/remitting course. Over time, pain may become refractory to treatment.

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Clinical features of lesions of the cerebral cortex

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1. lesions of the cerebral cortex often cause two main deficits : 1) contralateral motor or sensory deficits (depending on which region of the cortex is involved) and 2) aphasia
2. the hemiparesis seen with cortical lesions primarily affects the face arms and trunks. The legs may be affected, but typically not as severely because the neurons that control the LEs are in the intrahemispheric fissue (see homunculus diagram)
3. aphasia is common when the left hemisphere is involved. Visual-spatial deficits are more common when the right-hemisphere is involved

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Subcortical lesions - clinical/localizing features

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1. These involve the internal capsule (pure motor), cerebral peduncles, thalamus (pure sensory), and pons (clumsy hand, dysarthria)
2. The hemiparesis is usually complete (face, arm and leg) because the neurons controlling these structures all merge together subcortically and are very close together

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Cerebellar lesions- localizing/clinical features

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- incoordination, intention tremor, ataxia

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Brain stem lesions- localizing/clinical features

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- cranial nerve and spinal cord findings
- there is crossed hemiplegia-- deficit of the ipsilateral face and contralateral body because the corticospinal tract, dorsal columns and spinothalamic tracts cross but the cranial nerves do NOT

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Spinal cord lesions- localizing/clinical features

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- with acute injuries, spinal shock may be present and UMN signs may not be apparent initially
- the patient presents with UMN signs (spasticity, increased DTRs, clonus, positive Babinski's), but these signs may be present with lesions in the brainstem and cortical/subcortical regions as well
- there is a decrease in sensation below a sharp band in the abdomen/trunk. A pinprick is felt above the level but not below it. This is pathognomic for spinal cord disease -- the level of the lesion corresponds to the sensory level affected.

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Plexus (plexopathy)

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1. deficits (motor and sensory) involve more than one nerve. Findings are variable depending on which part of the plexus is involved
2. Trauma is the most common cause overall, especially in the brachial plexus. A post-surgical hematoma in the pelvis is a more common cause of lumbrosacral plexopathy
3. plexuses that commonly involved include - brachial plexus upper trunk C5-C6 and lower trunk C8-T11 (erb duchnne palsy), lumbrosacral (l5-s3)

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Radicular/Root Lesions- radiculopathy- clinical/localizing features

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1. pain is the key finding
2. this affect a group of muscles supplied by a spinal root (myotome) and sensory area supplied by a spinal root (dermatome). Therefore, the distribution of affected areas can help differentiate this from a peripheral neuropathy or a plexopathy
3. patients may present with weakness, atrophy, and sensory deficits in a dermatomal pattern. May have fasciculations and diminished DTRs

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Peripheral neuropathy - localizing/clinical features

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1. weakness is more prominent distally at the outset (as opposed to muscle myopathy which is more proximal) -- usually asymmetric
2. presents with diminished DTRs- may include sensory changes (numbness, paresthesias, tingling)
3. Can be due to diabetes (nerve infarction), trauma, entrapment or vasculitis
4. Common neuropathies include radial/ulnar/median/musculocutaneous nerves, long thoracic n, axillary n., common peroneal/fibular n, and femoral n.

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Neuromuscular junction lesions/problems - clinical/localizing features

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1. fatigability is a key finding-- think of Myasthenia Gravis. muscles become weaker with use and recover with rest
2. normal sensation, no atrophy

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Myopathy- clinical features

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1. myopathy refers to an acquired disease (dystrophy refers to inherited diseases)
2. symmetric weakness affects proximal muscles more than distal muscles (as opposed to neuropathies)- shoulders and hips are commonly affected
3. presents with normal reflexes, but these may diminish late in the disease in comparison to muscle weakness
4. normal sensation, no fasciculations (UMN sign)
5. muscle atrophy may occur later in the disease to disuse (in contrast to rapid atrophy in motor neuron disease)