Gen Path - Class 18

Front 1

What are two cranial nerve pathologies?

Back 1

(1)Bell’s Palsy, (2)Trigeminal Neuralgia

Front 2

What is Bell's palsy?

Back 2

Idiopathic facial paralysis. The most common cause of facial paralysis – no relation to stroke! Unilateral – Facial N. (CN VII). Possible viral connection (Viral meningitis or HSV 1?).

Front 3

What is the pathogenesis of Bell's palsy?

Back 3

Inflammation -- compression (within the auditory canal?) -- segmental demyelination at first -- with continued inflammation -- axonal degeneration

Front 4

What are three clinical manifestations of Bell's palsy?

Back 4

(1)Rapid onset, usually overnight; may be preceded by severe pain over the mastoid and/or fullness in the ear, (2)Asymmetrical facial appearance, (3)Possible decrease in taste, hearing, and lacrimation (tears)

Front 5

What are the risk factors of Bell's palsy?

Back 5

Increased risk with pregnancy and DM

Front 6

What is trigeminal neuralgia?

Back 6

‘Tic douloureux’. Intense paroxysms of lancinating pain of CN5. Not that common/ mostly in women 50-70. Usu. Unilateral, maxillary division of the nerve. No loss of sensation or motor control.

Front 7

What are four potential causes of trigeminal neuralgia?

Back 7

(1)Herpes zoster, (2)MS, (3)vascular lesions, (4)tumours

Front 8

What is the pathogenesis of trigeminal neuralgia?

Back 8

demyelinated fibers become hyperexcitable – intense pain to the lightest touch

Front 9

What are clinical manifestations of trigeminal neuralgia?

Back 9

Sudden onset, sharp/stabbing/severe; ‘like a lightening bolt’. Sx’s can occur in clusters – time between flare-ups will get progressively shorter, attacks more frequent with progression. Triggers: light touch, chewing, smiling, wind

Front 10

What are four metabolic neuropathies?

Back 10

(1)Diabetic, (2)Alcoholic, (3)Chronic renal failure, (4)Anemia

Front 11

What is a diabetic neuropathy?

Back 11

Progressive disorder in DM – nerve degeneration and atrophy. Peripheral or ANS nerves. Changes in microcirculation (endoneurial microvascular thickening) -- ischemia -- nerve damage (axonal degeneration)

Front 12

What is the etiology of diabetic neuropathy?

Back 12

chronic metabolic imbalance – caused by hyperglycemia and its consequences. Increased intracellular glucose in nerves -- Extra glucose shunted into the polyol pathway and converted to sorbitol and fructose -- decreased membrane Na+/K+ -ATPase activity, impaired axonal transport, and structural breakdown of nerves -- altered function of peripheral nerves.

Front 13

What is the result of increased production of free radicals?

Back 13

Increased production of free radicals damage to blood vessels leading to nerve ischemia. Antioxidant alpha-lipoic acid may hold promise for improving neuropathic symptoms.

Front 14

What are four clinical manifestations of diabetic neuropathy?

Back 14

(1)Insidious onset, (2)Sensory loss, (3)Motor loss, (4)May have autonomic involvement

Front 15

Describe the sensory loss of a diabetic neuropathy.

Back 15

‘stocking and glove’ pattern. Impaired vibration, proprioception, touch, pressure – like ‘walking on cotton balls’. Burning, prickling pain, tingling, electric shock–like feelings, aching, tightness, or hypersensitivity to touch.

Front 16

Describe the motor loss of a diabetic neuropathy.

Back 16

Loss of achilles DTR’s. Mild weakness is most common (as opposed to sign. mm weakness)

Front 17

Describe the autonomic involvement of a diabetic neuropathy.

Back 17

Autonomic neuropathies (ataxia, gait instability, or near syncope/syncope). In addition, autonomic neuropathies have further symptoms that relate to the anatomic site of nerve damage—gastrointestinal, cardiovascular, bladder, or sudomotor (sweating etc).

Front 18

How is diabetic neuropathy managed?

Back 18

Control hyperglycemia. Some medications. B-vitamins (particularly B12: methylcobalamin). Antioxidants – Ex. Alpha Lipoic Acid. Support of healthy vasculature. Early foot care.

Front 19

What is an alcoholic neuropathy?

Back 19

Alcohol in excess is neurotoxic. Chronic, long-term exposure to ethanol and its metabolites (direct damage to nerves) + nutritional deficiencies (thiamine and other B-vitamins). Total lifetime accumulation of ethanol is main predictor. Segmental demyelination -- axonal degeneration -- distal to proximal

Front 20

What are six clinical manifestations of an alcoholic neuropathy?

Back 20

(1)Loss of muscle, (2)pain/paresthesias/numbness (‘stocking & glove’), (3)dcr’d DTR’s, (4)diminished grip strength, (5)foot-drop gait, (6)Wernicke-Korsakoff Syndrome

Front 21

What are two potential results of a thiamine deficiency?

Back 21

(1)Wernicke’s encephalopathy, (2)Korsakoff’s Syndrome

Degeneration in hypothalamus , midbrain and mamillary bodies. 2 syndromes after often combined and called Wernicke-Korsakoff syndrome.

Front 22

What is Wernicke’s encephalopathy?

Back 22

Includes disturbances in ocular function, gait and mental function

Front 23

What is Korsakoff’s Syndrome?

Back 23

Mental deterioration where patients lose memory and makeup incredible stories (confabulation)

Front 24

What is chronic renal failure?

Back 24

CNS and PNS dysfunction from uremia. Uremia -- atrophy & demyelination of both sensory and motor peripheral nerves. Lower extremities much more commonly affected. May see restless leg syndrome at rest. [CNS effects: recent memory loss, inability to concentrate, perceptual errors, decreased alertness]

Front 25

How does anemia affect neurological disorders?

Back 25

Early stages of pernicious anemia (B12 deficiency) -- neuropathy. Uncoordinated movements and sensorimotor peripheral neuropathy with signs of spinal cord disease. Pathogenesis: degeneration of spinal cord. Severe pernicious anemia -- CNS sx’s [HA, drowsiness, dizziness, fainting, apathy, slow thinking, irritability, depression, etc.]

Front 26

What are four clinical manifestations of anemic neurological disorders?

Back 26

(1)Abnormal gait, (2)Symmetric, sensory sx’s, usually starts in feet and lower legs; dcr’d fine motor control of hands/fingers (might see deterioration in handwriting), (3)m/b moderate pain or paresthesias, (4)Loss of proprioception; +Romberg sign

Front 27

What are four infections/inflammations neuropathies?

Back 27

(1)Guillain-Barre Syndrome, (2)Post-polio syndrome, (3)Herpes Zoster/ Post-herpetic Neuralgia

Front 28

What is Guillain-Barre syndrome?

Back 28

Rapidly progressive neurological disorder assoc’d with infection. Bacterial: Campylobacter jejuni. Viral: Haemophilus influenza, EBV, CMV. Might be caused by the HPV vaccine??

Front 29

What is the pathogenesis of Guillain-Barre syndrome?

Back 29

Immune-mediated. AB attacks Schwann cells -- complement -- WBC infiltration -- macrophages -- inflammation -- demyelination (macrophages seem to strip myelin from nerves) -- axon degeneration

Front 30

What are seven clinical manifestations of Guillain-Barre syndrome?

Back 30

(1)Onset to peak impairment = 4 weeks or less = ‘classic’, (2)Rapidly ascending symmetric motor weakness, (3)Distal sensory impairment (usually paresthesia in toes), (4)Weakness rapidly spreads to arms, trunk, face, (5)Flaccid paralysis + no DTR’s, (6)Sometimes ANS sx’s: tachycardia, change to BP, (7)30% with GBS require mechanical ventilation

Front 31

What is Post-Polio Syndrome (PPS)?

Back 31

Polio has drastically declined, but there are still those who still experience new neuromuscular sx’s that occur decades after acute paralytic polio. Avg. = 25 years after initial infection. Unique to Polio: only motor impairments (focal and asymmetric); not the typical distal-to-proximal symmetric motor & sensory losses that we’ve seen in the other neuropathies

Front 32

What are the three types of polio?

Back 32

(1)Asymptomatic, (2)Non-paralytic infection – no worse than the flu, (3)Paralytic

Front 33

What is paralytic polio?

Back 33

Starts like the flu, but virus invades motor cell bodies. Fewer than 1% of all polio. Severity depends on degree of anterior horn cell involvement. 30% recovered/30% had mild residual sx’s/ 30% had paralysis. Recovery due to anterior horn cells recovering, plus collateral sprouting from intact peripheral nerves, and hypertrophy of spared muscle fibers.

Front 34

What is the pathogenesis of polio?

Back 34

Degeneration of the re-innervated muscles; this degeneration seems to happen more rapidly in people who had acute paralytic polio, than the normal rate of simple aging. Nervous system seems to no longer metabolically support the collateral nerves that formed in recovery.

Front 35

What are seven clinical manifestations of polio?

Back 35

(1)dcr’d muscle strength, (2)myalgia, (3)bulbar problems (respiratory and swallowing), (4)xs fatigue, (5)dcr’d endurance, (6)vasomotor abnormalities, (7)ADL’s affected

Front 36

What are the three most common signs of polio?

Back 36

(1)dcr’d muscle strength, (2)myalgia, (3)bulbar problems (respiratory and swallowing)

Front 37

What is Herpes Zoster/Post-Herpetic Neuralgia?

Back 37

Reactivation of Varicella-zoster virus. Initial infection: chicken pox. Virus lays dormant in sensory ganglia of CN’s and spinal nerves. Later activation = Herpes Zoster/Shingles. Reactivation -- inflammation in ganglia -- spreads along cranial/peripheral nerves -- segmental demyelination degeneration.

Front 38

What are five clinical manifestations of shingles?

Back 38

(1)Pain & tingling in the affected dermatome (Most common: Trigeminal & Thoracic), (2)Rash -- vesicles/blisters -- eventually burst, then crust, (3)Approx. 1 month to resolution – but can develop postherpeticneuralgia -- up to several months, (4)Pain – can be severe – burning, aching, cutting, stabbing -- hyperirritability of pain receptors, (5)Occas. affect motor neurons -- LMN sx’s

Front 39

What are two toxins that can lead to neuropathies?

Back 39

(1)Lead, (2)Pesticides/Organophosphates

Front 40

Describe a lead neuropathy.

Back 40

Paint (pre-1925); ceramics; car batteries; contaminated water; moonshine; 3rd world countries (gasoline still leaded); plastic miniblinds. Affects nerves to muscles in upper extremities. Wrist drop.

Front 41

Describe a Pesticides/Organophosphates neuropathy.

Back 41

Parathion (insecticide) is MCC of accidental poisoning &death. Inhibit cholinesterase activity -- acute cholinergic crisis -- over-stimulation of the neuromuscular junction. N/V, diarrhea, muscle fasciculations, weakness, paralysis (including respiratory), convulsions. Peripheral nerve involvement within 1-4 d. of exposure. Death from vasomotor collapse 2° to resp. paralysis

Front 42

What are two motor end-plate disorders?

Back 42

(1)Myasthenia gravis, (2)Botulism

Front 43

What is myasthenia gravis?

Back 43

MC disorder of neurotransmitter affecting neuromuscular fxn

Front 44

What is the etiology and risk factors of myasthenia gravis?

Back 44

Hyperthryoidism, DM, SLE, RA, women (age 20-40)

Front 45

What is the pathogenesis of myasthenia gravis?

Back 45

Autoimmune – Hypersensitivity Type II – antibodies block Ach (Acetylcholine) receptors at the NM junction

Front 46

What is the clinical manifestation of myasthenia gravis?

Back 46

Skeletal muscle weakness/fatigue; eyes usu. affected first; then neck and jaw

Front 47

What is botulism?

Back 47

Paralytic food poisoning -- neuropathy. Clostridium botulinum.

Front 48

What is the etiology of botulism?

Back 48

Neurotoxin produced by the bacteria – found in improperly preserved canned food; also key cause of wound infection.

Front 49

What are four clinical manifestations of botulism?

Back 49

(1)Malaise, (2)weakness, (3)blurred vision, (4)N/V, possibly fatal within 6-8 hours due to respiratory failure

Front 50

What is the pathogenesis of botulism?

Back 50

Toxin inhibits ACh release at NM junction -- flaccid paralysis.

Front 51

What is Complex Regional Pain Syndrome?

Back 51

aka: Reflex Sympathetic Dystrophy. Abnormal response in peripheral nerves. Develops after trauma. Pain is greater than expected for the degree of tissue trauma. Spreads from localized -- regional. Burning pain – spontaneous occurrence – doesn’t correspond with stimulus/trigger. Approx. 5% incidence after injury. Dx usually delayed.

Front 52

What are paraneoplastic neuropathies?

Back 52

Any portion of the nervous system can be compromised by cancer. Development of paraneoplastic neuropathy occurs subacutely or chronically over weeks to months, and precedes the discovery of the tumour from months to years. Nerve cell body is the 1° site of involvement. Large diameter neurons preferentially affected.

Front 53

What is the pathogenesis of paraneoplastic neuropathies?

Back 53

Possibly autoimmune response, initially directed against the cancer’s antigen, then attacks nerves

Front 54

What are two clinical manifestations of paraneoplastic neuropathies?

Back 54

(1)Numbness & paresthesias, (2)Burning, aching, or lancinating pain

Front 55

What is cephalgia?

Back 55

One of the most common symptoms seen in health care. Primary headaches or secondary headaches. Be suspicious with: Head trauma; seizures; rapidly changing sx pattern; meningitis; motor and/or sensory deficits

Front 56

What are three primary headaches?

Back 56

(1)Tension, (2)Migraine, (3)Cluster

Front 57

What is a secondary headache?

Back 57

Almost all diseases can have a symptom of headache!

Front 58

What is a tension headache?

Back 58

Most Common type of headache, adults 30-40. Etiology: Stress, anxiety, or poor cervical posture. Chronic, bilateral, gradual onset; progressively more frequent. Stress, anxiety, or poor cervical posture. Muscle spasms -- changes in neurotransmitters & brain fluids.

Front 59

What are the two types of tension headaches?

Back 59

(1)Episodic tension headache, (2)Chronic tension headache

Front 60

What is a episodic tension headache tension headache?

Back 60

Associated with a stressful event. Moderate intensity, self-limited, and usually responsive to nonprescription drugs.

Front 61

What is a chronic tension headache tension headache?

Back 61

Often recurs daily. Associated with contracted muscles of the neck and scalp. Bilateral and usually occipitofrontal

Front 62

What is the pathogenesis of a tension headache?

Back 62

Not well understood. Multifactoral and may include abnormal neuronal sensitivity and pain facilitation, not only abnormal muscle contraction

Front 63

What are clinical manifestations of a tension headache?

Back 63

Chronic, bilateral, gradual onset; progressively more frequent

Front 64

What is a cluster headache?

Back 64

Neurological disorder. Repeated, extremely painful in the same area of head - Occur in ‘clusters’ – lasting several days to weeks, then can disappear for months or years.

Front 65

What are five potential causes of cluster headaches?

Back 65

(1)Unknown, (2)Genetics, (3)Smoking, (4)Alcohol, (5)Hypothalamus dysfunction

Front 66

What is the pathophysiology of cluster headaches?

Back 66

Not fully understood. Current theories implicate mechanisms such asvascular dilation, trigeminal nerve stimulation, and circadian effects. Histamine release, an increase in mast cells, genetic factors, and autonomic nervous system activation may also contribute.

Front 67

What are six clinical features of cluster headaches?

Back 67

(1)Extreme pain - excruciating pain (has been known to lead to suicide), (2)Rapid, (3)Unilateral, (4)Usually only last up to 2 hours, (5)Reoccurring for a few days, (6)Regional autonomic changes (lacrimation, nasal discharge, flushed skin, sweating)

Front 68

What is a migraine headache?

Back 68

Vascular disorder. Affects women more than men.

Front 69

What are three potential causes of migraine headaches?

Back 69

(1)Triggers – food, stress, menstruating, environmental factors, (2)Genetics, (3)Possible role of serotonin – can increased platelet activity and increased pain sensitivity

Front 70

What is the pathogenesis of a migraine headache?

Back 70

Prodromal vasoconstriction of cerebral blood flow -- hypoxia -- then vasodilation + neurotransmitter changes

Front 71

What are three clinical manifestations of migraine headaches?

Back 71

(1)Prodrome: ‘aura’; short period of visual disturbance, depression, anxiety, anorexia, dizziness, paresthesias (hands &face mostly), (2)HA: unilateral or bilateral; N/V; photophobia; usually starts behind the same eye, and has a typical time frame for each person (hours to days), (3)Postdrome phase (Sore where headache occurred, Impaired thinking)

Front 72

What is schizophrenia?

Back 72

Schizophrenia affects 1% of the Canadian population. Mental disorder characterised by a breakdown in thinking and poor emotional responses.

Front 73

What are three characteristics of schizophrenia?

Back 73

(1)Hallucinations, (2)Delusions, (3)Disorganized thought process

Front 74

What are two potential causes of schizophrenia?

Back 74

(1)Genetics, (2)Environmental – substance abuse

Front 75

What is the pathogenesis of schizophrenia?

Back 75

Misfiring of dopaminergic neurons (Meds that block dopamine -- decrease in symptoms). Reduction in brain tissues (Frontal lobes,Hippocampus, Temporal lobes)

Front 76

What is bipolar disorder?

Back 76

Aka. Manic depression. Mental illness characterised by episodes of elevated mood or agitated mood alternating with episodes of depression.

Front 77

What are five potential causes of bipolar disorder?

Back 77

(1)Unknown, (2)Genetic, (3)Brain activity, (4)Brain trauma, stroke, (5)Dopamine

Front 78

What is depression?

Back 78

Usually refers to Major depressive disorder.

Front 79

What are three types of depression?

Back 79

(1)Dysthymia, (2)Post-partum depression, (3)Premenstrual Dysmorphic Disorder

Front 80

What is dysthymia?

Back 80

Persistent mild depression

Front 81

How does depression differ from bereavement?

Back 81

sx persist for > two months or show marked functional impairment, morbid preoccupation with worthlessness, suicidal ideation, psychotic symptoms, or psychomotor retardation.

Front 82

What is the pathophysiology of depression?

Back 82

Not been clearly defined. Current evidence points to a complex interaction between neurotransmitter availability and receptor regulation and sensitivity underlying the affective symptoms.