Medstudy Neurology

Front 1

Elevated maternal alpha-fetoprotein, girls more commonly affected, failure of closure of anterior neural tube

Back 1

Anencephaly

Front 2

Herniation of the brain and/or its coverings through a skull defect (75% occipital region), males and females equally affected, may see elevated alpha-fetoprotein

Back 2

Encephalocele

Front 3

CNS infections (mumps, meningitis), intrauterine CMV, rubella, toxoplasmosis, and syphilis, and subarachnoid hemorrhages can lead to this

Back 3

Communicating Hydrocephalus

Front 4

Posterior fossa cyst continuous with 4th ventricle, partial or complete absence of cerebellar vermis, and hydrocephalus

Back 4

Dandy-Walker Malformation

Front 5

Downward displacement of brainstem and cerebellum, impaired spinal fluid flow, associated with spina bifida and meningomyelocele

Back 5

Chiari Malformation

Front 6

Headache, esp in early morning, nausea, vomiting, 3rd and 6th CN palsies, papilledema

Back 6

Signs of Hydrocephalus

Front 7

Paracentral cavity (syrinx) in the spinal cord, wasting of small muscles of the hand, sensory deficits of arms, absence of DTRs, bilateral loss of pain and temp (if lateral spinothalamic tract injury) leading to trophic ulcers of fingertips

Back 7

Syringomyelia

Front 8

Short neck, limited neck motion, and low occipital hairline
a. majority of cervical and upper thoracic vertebrae fused
b. complete segmentation fails to occur at 1-2 spaces
c. lesions occur as above but also at lower thoracic and lumbar vertebrae

Back 8

Klippel-Feil Syndrome
a. Type I
b. Type II
c. Type III

Front 9

Lissencephaly, prominent forehead, bitemporal "hollowing," anteverted nostrils, prominent upper lip, micrognathia

Back 9

Miller-Dieker Syndrome

Front 10

Group of disorders that occur due to a CNS insult during early development that results in chronic, non-progressive afflictions of movement, posture, and tone

Back 10

Cerebral Palsy
Spastic 70%
Dyskinetic 15%
Ataxic 5%
Mixed 10%

Front 11

Impaired, willful, uncontrolled and purposeless movements that disappear during sleep. Most movement is choreoathetoid, pathology is in the basal ganglia

Back 11

Dyskinetic CP

Front 12

Dysfunction of coordination with gait, wide based gait, unable to perform finger to nose well, due to cerebellar damage

Back 12

Ataxic CP

Front 13

1. Transient postictal hemiparesis, EEG activity consistent with seizures, no acute infarction on MRI
2. Complicated migraine with focal deficits, + family history, no infarction on MRI
3. Alternating hemiplegia, onset in <2 yrs of age, +seizures

Back 13

Ddx for Acute Hemiplegia

Front 14

Chronic, occlusive, cerebrovascular disease associated with sickle cell, NF type 1, and cranial radiation, refers to extensive collateral vessels resulting from prior occlusions of arteries around the circle of Willis

Back 14

Moyamoya Disease

Front 15

Deficiency of alpha-galactosidase causing accumulation of ceramide trihexoside in vascular endothelium that leads to arterial narrowing, ischemia, and infarction

Back 15

Fabry Disease

Front 16

Usually asymptomatic, associated with coarctation of the aorta, polycystic kidneys, Ehlers-Danlos syndrome, and Marfan syndrome, arise from internal carotid artery or anterior cerebral artery

Back 16

Saccular Aneurysms

Front 17

Virus associated with infarcts in basal ganglia or internal capsule

Back 17

Varicella

Front 18

Bilateral orbital ecchymoses or posterior auricular ecchymoses, otorrhea, and/or rhinorrhea are indicative of this

Back 18

Basilar Skull Fracture

Front 19

Worry about this in a patient who is unconscious by trauma, regains consciousness over several hours, then worsens

Back 19

Epidural Hematoma

Front 20

Early herniation characterized by dilated pupil on side of hematoma progressing to complete 3rd nerve palsy and contralateral hemiplegia, late stages both pupils become fixed and dilated, respiration is slow and irregular, and hypotension and tachycardia develop

Back 20

Epidural Hematoma with Herniation

Front 21

Most common cause of peripheral nerve injury

Back 21

Birth Trauma

Front 22

Sagging shoulder, arm hanging limp and internally rotated, pronated wrist - due to paralysis of spinate, deltoid, biceps, brachioradialis, and extensor carpi radialis muscles

Back 22

Erb-Duchenne Type Injury (Upper Plexus Root)

Front 23

Sensory and vasomotor involvement with paralysis of flexors and extensors of the forearm and intrinsic muscles of the hand, if 1st thoracic root is involved can have Horner syndrome and cervical sympathetic damage

Back 23

Klumpke-Dejerine Type Injury (Lower Plexus Root)

Front 24

Short duration, rapid, bilateral, symmetric muscle contractions, isolated or repetitive jerks, possibility of falling, best treated with valproate or other broad-spectrum anti-convulsants

Back 24

Myoclonic Seizures

Front 25

Morning myoclonic jerks, +/- hx of absence seizures, generalized tonic-clonic seizures occurring just after awakening or during sleep, normal intelligence, onset at age 8-20, +family history, valproate is drug of choice

Back 25

Juvenile Myoclonic Epilepsy

Front 26

Brief, abrupt onset and end, onset between 3 and 12 yrs, atypical forms can have brief jerks of eyelids and limbs, transient change in tone, not associated with auras, treat with ethosuximide, lamotrigine, or valproate

Back 26

Absence Seizures

Front 27

Focal motor findings including asynchronous clonic or tonic movements, no loss of consciousness or post-ictal state, may be followed by Todd paralysis

Back 27

Simple Partial Seizures

Front 28

Altered consciousness or unresponsiveness, automatisms, due to abnormalities in limbic structures especially mesial temporal sclerosis, treat with carbamazepine, oxcarbazepine, or phenytoin

Back 28

Complex Partial Seizures

Front 29

2 treatments for infantile spasms

Back 29

ACTH (most common)
Vigabatrin

Front 30

Severe seizures, mental retardation, generalized, bilateral synchronous, sharp-wave and slow-wave complexes occurring in repetitive fashion, poor prognosis, poor control with drug therapy

Back 30

Lennox-Gastaut Syndrome

Front 31

Initial generalized seizures that become focal, unremitting, limited to one part or side of the body, hemiparesis, diminished intelligence, and hemianopia occur, 70% have hx of preceding infection or inflammatory process

Back 31

Rasmussen Syndrome

Front 32

Preferred treatment for partial and secondary generalized seizures

Back 32

Carbamazepine, oxcarbazepine, or topiramate

Front 33

Preferred treatment for absence seizures

Back 33

Ethosuximide, lamotrigine, or valproic acid

Front 34

Preferred treatment for idiopathic generalized tonic-clonic seizures

Back 34

Valproate, topiramate, or carbamazepine - contraindicated if spike wave pattern on EEG

Front 35

Preferred treatment for complex partial seizures

Back 35

Carbamazepine, phenytoin, or oxcarbazepine

Front 36

Side effects include hepatotoxicity, pancreatitis, weight gain, hair loss, thrombocytopenia, increased risk of teratogenicity

Back 36

Valproic Acid

Front 37

Side effects include leukopenia, hepatotoxicity, dizziness, drowsiness, diplopia, and SIADH, avoid erythromycin

Back 37

Carbamazepine

Front 38

Side effects include hirsutism, gum hypertrophy, ataxia, skin rash, nystagmus, drowsiness, blood dyscrasias, and Stevens-Johnson syndrome

Back 38

Phenytoin

Front 39

Side effects include abdominal pain, skin rash, liver dysfunction, and leukopenia

Back 39

Ethosuximide

Front 40

Side effects include somnolence, vomiting, ataxia, rash, nystagmus, and hyponatremia

Back 40

Oxcarbazepine

Front 41

PLEDS (periodic lateralizing epileptiform discharges) are characteristic of this

Back 41

HSV

Front 42

Common infections that result in higher incidence of febrile seizures

Back 42

Roseola (HHV6) and Shigella

Front 43

Occur at elast 5 times, last 2-72 hrs without identifiable etiolog and 2 of the following: pain on one side, pulsating/throbbing character, mod-severe intensity, increasing severity with activity

Back 43

Criteria for Migraine

Front 44

Paroxysmal, repeated episodes of hemiplegia lasting minutes to days, associated with intellectual decline, occurs in those <18 months

Back 44

Alternating Hemiplegia of Childhood

Front 45

Occurs most often in adolescent girls, presents with vertigo, syncope, and dysarthria. May alsoh ave visual alterations or loss of consciousness

Back 45

Basilar Artery Migraine

Front 46

Gene located on chromosome 5q, present with hypotonia, weakness of intercostal muscles, muscle atrophy, and fasiculations. Lesion is degeneration of anterior horn cell and also bulbar nuclei

Back 46

Spinal Muscular Atrophy

Front 47

Presents at < 6 months of age, hypotonia, weakness, difficulty feeding, tongue fasiculations. Most pts die by 2 years of age

Back 47

Type I SMA or Werdnig-Hoffman

Front 48

Healthy at birth, lose normal milestones by age 2, weakness is static but progresses with intercurrent illness, majority die by age 12.

Back 48

Type II SMA

Front 49

Presents between ages 2 and 17, unable to stand or walk unaided, degree of deficit correlates with age of onset

Back 49

Type III SMA or Kugelberg-Welder

Front 50

X-linked recessive, mutation in dystrophin gene. Present between 2-6 years with frequent falling, waddling gait, toe walking, calf muscle pseudohypertrophy, Gower's sign

Back 50

Duchenne Muscular Dystrophy

Front 51

Postsynaptic receptors for acetylcholine are reduced in number, resulting in the postjunctional membrane being less sensitive to acetylcholine

Back 51

Myasthenia Gravis

Front 52

Exposure to transplacental passage of maternal acetylcholine receptor antibodies, present with hypotonia, weak cry, difficulty feeding, facial weakness, palpebral ptosis, respiratory compromise. Resolves 2-12 wks after antibodies clear

Back 52

Neonatal Myasthenia Gravis

Front 53

AR disorder, variable age in onset, do NOT have circulating antibodies to acetylcholine receptor

Back 53

Congenital Myasthenia Gravis

Front 54

Acquired autoimmune disorder, girls > boys, autoantibodies to acetylcholine receptors, progresses with worsening muscle weaknes and respiratory compromise, ocular muscles are involved

Back 54

Juvenile Myasthenia Gravis

Front 55

Diagnostic test for myasthenia gravis

Back 55

Edrophonium (Tensilon) test or neostigmine

Front 56

Weakness starting distally and moving proximally, can involve respiratory muscles and CN, rare sensory losses, DTRs absent, no Babinski sign, autonomic instability, papilledema, and ataxia, LP reveals high protein

Back 56

Guillain-Barre Syndrome (AIDP)

Front 57

Infections more likely to be associated with Guillain-Barre Syndrome

Back 57

Campylobacter, Mycoplalsma, and EBV

Front 58

Constipation, generalized weakness, decreased ability to suck, poor gag reflexes, absent DTRs, facial diplegia, ptosis, dry mouth, lack of pupillary response to light, complete lack of muscle/head control

Back 58

Infantile Botulism

Front 59

Dryness, sore mouth, blurry vision, double vision, nausea, vomiting, anhidrosis, ophthalmoplegia, symmetrical facial, bulbar, and limb abnormalities, paralysis of pupils

Back 59

Botulism (older children)

Front 60

Treatment for botulism

Back 60

Antitoxin and Guanidine

Front 61

Onset between 2-6 yrs, 50% have prior URI or GI illness, ataxia, hypotonia, tremor, horizontal nystagmus, dysarthria, normal sensory exam, normal DTRs, resolves in 6-8 weeks

Back 61

Acute Cerebellar Ataxia

Front 62

Infections associated with acute cerebellar ataxia

Back 62

VARICELLA, rubeola, mumps, rubella, echoviruses, EBV, influenza, group A strep, salmonella

Front 63

Segmental spinal cord disease with both motor and sensory abnormalities at and below the level of the lesion, severe back pain that radiates to front followed by progressive paraparesis, loss of sphincter tone, lost of pain and temperature sensation

Back 63

Transverse Myelitis

Front 64

Vision abnormalities, especially optic neuritis, oculomotor disturbance, incoordination, sensory deficits, increase of IgG or oligoclonal bands in CSF

Back 64

Multiple Sclerosis