Neurology Flashcards

1
Q

arbovirus pattern

A

warm climates, carried by insects (west nile, St. Louis)

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2
Q

enterovirus pattern

A

generalized neurological findings, transmitted human to human

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3
Q

herpes encephalitis diagnostic test

A

DNA PCR

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4
Q

frontal headache with bandlike pressure

A

stress/tension headache

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5
Q

episodic severe headaches that cause a child to stop their activities and lay down

A

migraine

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6
Q

migraine headache treatment

A

ibuprofen, Tylenol, fluids and rest

ergotamine and sumatriptan if needed

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7
Q

migraine headache prevention

A

cyproheptadine or topiramate

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8
Q

contraindicated when signs of increased ICP

A

LP

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9
Q

diagnostic test of choice when concerned for increased ICP

A

CT with contrast urgently, MRI once stable

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10
Q

increased ICP of unknown etiology aka

A

psuedotumor cerebri

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11
Q

untreated pseudotumor cerebri leads to

A

papilledema –> optic disk atrophy –> blindness

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12
Q

double vision in pseudo tumor cerebri is 2/2 to

A

6th nerve palsy

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13
Q

headache in pseudotumor cerebri gets worse with

A

laying flat and valsalva

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14
Q

common cause of pseudotumor cerebri

A

megadose vitamin intake, especially vitamin A

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15
Q

eye findings in infants with increased ICP

A

setting sun/downward deviation

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16
Q

medications that can cause pseudotumor cerebri

A

vitamin A, steroids, thyroxine, lithium, some antibiotics

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17
Q

psudotumor cerebri treatment

A

acetazolamide (carbonic anhydrase inhibitor), severe cases require steroids and surgery/shunt

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18
Q

Cushing’s triad

A

HTN, bradycardia, abnormal respirations (late findings of increased ICP/impending herniation)

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19
Q

major risk factor for hydrocephalus

A

neural tube defect (chair malformation or myelomeningocele)

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20
Q

most shunt infections occur when

A

within 6 months of placement

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21
Q

presentation of shunt malfunction

A

symptoms of increased ICP but without fever

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22
Q

common location of brain tumors in kids

A

posterior fossa

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23
Q

most common type of solid tumors in kids

A

brain tumors

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24
Q

contrast enhancing 4th ventricle mass

A

medulloblastoma

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25
Q

most common malignant brain tumor of childhood

A

medulloblastoma

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26
Q

medulloblastoma presentation

A

headache, ataxia, obstructive hydrocephalus

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27
Q

presentation of posterior fossa tumors

A

headache, ataxia, head tilt, torticollis, vomiting

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28
Q

supratentorial tumors

A

cranioopharyhngioma, optic nerve glioma

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29
Q

craniopharyngioma presentation

A

slow progression with triad of endocrinopathies (DI, short stature), visual disturbances and headaches

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30
Q

cause of chronic visual field deficits in craniopharyngioma

A

pressure on the optic tracts

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31
Q

cause of hydrocephalus in craniopharyngioma

A

obstruction of the 3rd ventricle

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32
Q

craniopharyngioma treatment

A

surgery +/- radiation

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33
Q

optic nerve gliomas have a 25% association with

A

neurofibromatosis

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34
Q

patients with what disease have an increased risk for brain abscess

A

pulmonary, sinus and cyanotic heart disease

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35
Q

CT description of brain abscess

A

ring enhancing lesion

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36
Q

most common cause of sinusitis

A

S. aureus

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37
Q

common treatment for brain abscess

A

nafcillin/vancomycin, metronidazole and ceftriaxone

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38
Q

cause of cranial bruit in a neonate with hydrocephalus and CHF

A

vein of Galen malformation

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39
Q

presentation of younger patients with Wilson’s disease

A

liver disease

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40
Q

presentation of older patients with Wilson’s disease

A

neuro/psych symptoms

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41
Q

diagnosis with acute hepatic failure, dystonia and mental status changes

A

wilson’s disease

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42
Q

sudden drops precipitated by sudden emotion such as laughter

A

cataplexy

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43
Q

diagnosis of narcolepsy

A

overnight polysomnography and sleep latency test

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44
Q

acute onset ataxia is most likely 2/2

A

post-vital

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45
Q

ataxia telangiectasia findings

A

CNS/sometimes intellectual disability, skin and eye findings from capillary dilations, decreased IgA, IgG and T-cell function causing frequent upper and lower respiratory tract infections

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46
Q

advanced ocular telangiectasias cause

A

inability to voluntarily make rapid eye movements (pupillary reflexes and acuity usually are normal)

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47
Q

high risk of what with ataxia telangiectasia

A

malignancy - especially Hodgkin lymphoma and leukemia

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48
Q

ataxia telangiectasia inheritance

A

autosomal recessive

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49
Q

Friedrich ataxia inheritance

A

autosomal recessive

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50
Q

friedreich ataxia presentation

A

slow and clumsy gait in late childhood or early adolescence

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51
Q

cause of ataxia in friedreich ataxia

A

cerebellar component and loss of proprioception

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52
Q

clinical features that distinguish Friedreich ataxia

A

elevated plantar arch, no lower extremity DTRs, cardiomyopathy leading to CHF

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53
Q

relatively rapid random repetitive purposeless movements

A

chorea

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54
Q

part of the brain most affected in movement disorders

A

basal ganglia

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55
Q

Sydenham chorea association

A

major Jones criteria for rheumatic fever

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56
Q

Huntington’s disease triad

A

chorea, hypotonia and emotional lability

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57
Q

juvenile Huntington’s disease presents most often with

A

rigidity rather than hypotonia

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58
Q

chorea treatment

A

dopamine blocking agents ex: haloperidol (also can use fluphenazine, risperidone or tetrabenazine)

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59
Q

treatment of dystonic reaction

A

diphenydramine

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60
Q

neck hyperextension and decreased extra ocular movements

A

dystonic reaction

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61
Q

meds that can cause dystonic reactions

A

neuroleptics ex: metoclopramide or promethazine

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62
Q

choreiform movements vs tics

A

choreiform movements get worse during purposeful movement and cannot be voluntarily suppressed whereas tics improve during purposeful movements and can be suppressed

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63
Q

Tourette syndrome diagnosis length

A

1 year of symptoms

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64
Q

location of lesion with weak eye movements, ipsilateral facial weakness and contralateral body weakness

A

brainstem lesion

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65
Q

location of lesion with loss of motor and sensation, lord of bladder/bowel, increased reflexes

A

spinal cord lesion

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66
Q

test if concerned for spinal cord lesion

A

MRI spine

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67
Q

acute transverse myelitis aka

A

post infectious myelitis

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68
Q

cause of acute transverse myelitis

A

lymphocytic infiltration and demyelination of the nerves/spinal cord 2/2 inflammation

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69
Q

CSF findings in acute transverse myelitis

A

increased polys and negative gram stain

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70
Q

MRI findings in acute transverse myelitis

A

swelling of the spinal cord

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71
Q

main concern in acute transverse myelitis

A

respiratory compromise/arrest

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72
Q

test that must be preformed first with acute transverse myelitis

A

MRI before LP

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73
Q

abrupt onset of weakness, hypotonia and decreased reflexes followed by increased tone and hyperreflexia

A

acute transverse myelitis

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74
Q

fever and sudden onset of paralysis

A

acute transverse myelitis

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75
Q

treatment for extrinsic spinal cord mass lesion

A

high dose IV dexamethasone

76
Q

werdnig-hoffman disease aka

A

SMA type 1

77
Q

mechanism of werdnig-hoffman disease

A

degeneration of anterior horn cells - only motor function is affected

78
Q

diagnosis of wedding-hoffmann disease

A

muscle biopsy

79
Q

hypotonia, poor suck and tongue fasciculations

A

werdnig-hoffman disease (SMA type 1)

80
Q

finding that distinguishes SMA from botulism

A

tongue fasciculations

81
Q

when in development do neural tube defects occur

A

between weeks 3 and 4

82
Q

things that increase the risk for neural tube defects

A

maternal folate deficiency and maternal anticonvulsant use

83
Q

who needs consulted if you suspect neural tube defects in a newborn

A

ortho and urology

84
Q

early subtle findings of occult spinal dysraphism

A

lipoma, patch of hair, hemangioma, discoloration or sacral dimple

85
Q

late findings of occult spinal dysraphism

A

leg length discrepancy, elevated arches, gait abnormalities

86
Q

management for suspected spinal trauma

A

embolization and methylprednisolone 30 mg/kg over 1 hour

87
Q

acquired idiopathic unilateral facial paralysis 2/2 post viral inflammation of the 7th CN

A

bell’s palsy

88
Q

is forehead involved in bell’s palysy

A

includes forehead weakness (decreased wrinkling)

89
Q

unilateral facial weakness that spares the forehead

A

evaluate for stroke

90
Q

treatment for bell’s palsy

A

prednisone x5 days (works best if started within 3 days of symptom onset) or acyclovir in severe cases

91
Q

facial paralysis associated with painful vesicles in the ear canal

A

Ramsay hunt syndrome (reactivation of varicella zoster)

92
Q

progressive proximal motor weakness and areflexia that progresses from lower to upward symmetrically

A

Guillain barre syndrome

93
Q

what is always present in guillain barre syndrome

A

areflexia

94
Q

physical exam findings in Guillain barre

A

CN findings na dysautonomia (tachycardia, orthostatic hypotension, dizziness)

95
Q

CSF findings of Guillain barre sydrome

A

increased protein, normal cell count

96
Q

Guillain barre treatment

A

supportive plus plasmapheresis and IVIG

97
Q

autoimmune disorder with antibodies against the acetylcholine receptor in the neuromuscular junction

A

myasthenia gravis

98
Q

what presents similarly to Guillain barre

A

tick paralysis during the summer months

99
Q

characteristics of weakness with myasthenia gravis

A

weakness gets worse with activity and improves with rest

100
Q

common first presentation of myasthenia gravis

A

ptosis

101
Q

myasthenia gravis is associated with

A

thymoma

102
Q

test to confirm myasthenia gravis

A

tensilon test (uses edrophonium, a short acting acetylcholinesterase inhibitor)

103
Q

chronic treatment for myasthenia gravis

A

pyridostigmine (inhibits acetylcholinesterase to increase amount of acetylcholine available at the neuromuscular junction)

104
Q

treatment that sometimes works to cure myasthenia gravis

A

thymectomy

105
Q

MOA of botulism toxin

A

blocks the release of acetylcholine from the neuromuscular junction

106
Q

type of paralysis with botulism

A

descending paralysis

107
Q

cause of Duchenne muscular dystrophy

A

absence of dystrophin

108
Q

how to diagnose Duchenne muscular dystrophy

A

muscle biopsy

109
Q

what is more severe in Becker compared to Duchenne’s

A

cardiac involvement

110
Q

genetic testing for Duchenne muscular dystrophy

A

Xp21 gene

111
Q

finding in asymptomatic female carrier of Duchenne muscular dystrophy

A

elevated CPK

112
Q

myotonic muscular dystrophy affects what kind of muscle

A

smooth and striated muscles

113
Q

myotonic muscular dystrophy presentation

A

muscles that have slow relaxation after contraction, usually presents around 4 to 5 years old

114
Q

type of muscle wasting in myotonic dystrophy

A

distal

115
Q

myotonic muscular dystrophy inheritance pattern

A

autosomal dominant

116
Q

sudden electrical discharge of nerve cells from the gray matter that progresses to the white matter

A

seizure

117
Q

status epilepticus

A

seizure >30 minutes or repeat seizure without returning to normal in between

118
Q

age range for febrile seizures

A

6 months to 5 years

119
Q

simple febrile seizure

A

generalized seizure that lasts less than 15 minutes and only once within a 24 hour time period

120
Q

increased risk for developing epilepsy with febrile seizures if

A

first febrile seizure is complex

121
Q

complex febrile seizure

A

lasts longer than 15 minutes, recurs within 24 hours or focal

122
Q

brief staring spells/non responsive during this time without post octal period

A

absence seizure

123
Q

3 per second spike and wave on EEG

A

absence seizure

124
Q

diagnostic maneuver for absence seizure

A

hyperventilation

125
Q

absence seizure treatment

A

ethosuximide, lamotrigine or valproic acid

126
Q

prognosis with absence seizure

A

younger the child the better response to treatment

127
Q

monitoring that needs done with valproic acid

A

watch for thrombocytopenia, elevated LFTs and elevated pancreatic enzymes

128
Q

tonic phase

A

flexion of trunk, extension of back, arms and legs, no loss of bladder control, lasts ~30 seconds

129
Q

clonic phase

A

clonic convulsive movements with tremors, short periods of atonia where there is loss of bladder and apnea

130
Q

drug of choice for generalized tonic clonic seizures

A

valproic acid

131
Q

potential side effect of lamotrigine

A

Steven Johnson’s

132
Q

EEG and juvenile myoclonic epilepsy

A

usually normal

133
Q

juvenile myoclonic epilepsy presentation

A

myoclonic jerks upon waking, most have GTCs some have absence seizures`

134
Q

juvenile myoclonic epilepsy treatment

A

valproic acid or levetiracetam

135
Q

west syndrome aka

A

infantile spasms

136
Q

west syndrome triad

A

infantile spasms, hypsarrhythmia on EEG, developmental delay

137
Q

typical time of presentation with infantile spasms

A

3 to 9 months

138
Q

sudden flexion or extension of the body

A

infantile spasm

139
Q

association between infantile spasms and

A

tuberous sclerosis

140
Q

first line treatment of infantile spasms

A

ACTH (others are steroids, benzodiazepines, focal resection)

141
Q

prognosis with infantile spasms

A

depends on developmental status prior to spasms starting

142
Q

simple in seizures means

A

consciousness maintained

143
Q

partial in seizures means

A

focal

144
Q

simple partial seizure treatment

A

carbamazepine (many go away by adolescence or don’t need treatment)

145
Q

benign rolandic epilepsy aka

A

benign childhood epilepsy with centrotemporal spikes

146
Q

most common partial epilepsy of childhood

A

benign rolandic epilepsy

147
Q

self limited seizures involving the face

A

benign rolandic epilepsy

148
Q

benign rolandic epilepsy is associated with

A

migraines

149
Q

benign rolandic epilepsy most often occurs

A

at night

150
Q

treatment for benign rolandic epilepsy

A

only if 3 or more episodes

151
Q

prognosis of benign rolandic epilepsy

A

usually resolve by age 16

152
Q

benign Rolandic epilepsy during the ay

A

fully aware but unable to speak and have unilateral sensory involvement (usually one side of the tongue)

153
Q

benign rolandic epilepsy EEG findings

A

biphasic focal centrotemporal spikes and slow waves

154
Q

treatment of seizure if Ativan doesn’t work

A

fosphenytoin

155
Q

can teens with seizures drive

A

if on antieplieptic and seizure free x 12 months

156
Q

when can you try to stop antiepileptics

A

if seizure free x2 years

157
Q

most common cause of CP in preemies

A

perinatal infection

158
Q

CP with bilateral leg spasticity, delayed walking and tip toe walking

A

spastic diplegia

159
Q

CP with spasticity of one half of the body (arm, trunk and leg of that side)

A

spastic hemiplegia

160
Q

CP with increased tone in all 4 extremities (L>U)

A

spastic quadriplegia

161
Q

CP with increased tone in all 4 extremities, dystonia and strange movements

A

dyskinetic

162
Q

next step with CVA confirmed on CT

A

cerebral angiography

163
Q

history consistent with stroke and has elevated lactate and sensorineural deafness

A

mitochondrial disease - get a molecular analysis of DNA

164
Q

most common cause of hemorrhagic stroke in children

A

vascular malformations

165
Q

diagnosis of infant with sever seizures and CT showing bilateral clefts within the cerebral hemispheres

A

schizencephaly

166
Q

friederich ataxia chromsome

A

9

167
Q

diagnosis of newborn with herniation of the brain and its coverings through a skull defect in the occipital region

A

encephalocele

168
Q

diagnosis in newborn with herniation of meninges through defect in posterior vertebral arches, spinal cord is normal and area is well covered with skin

A

meningocele

169
Q

most common abnormalities that develop in kids with myelomeningocele

A

hydrocephalus and chiari II malformation

170
Q

diagnosis of newborn with severe seizures and CT shows brain without cerebral convolutions and poorly formed Sylvian fissure

A

lissencephaly

171
Q

diagnosis with prenatal brain US showing widely separated frontal horns w/ 3rd ventricle high between the lateral ventricles, mom has used cocaine

A

agenesis of the corpus callosum

172
Q

most likely prothrombotic factor to cause an ischemic stroke in a child

A

acquired antiphospholipid antibody

173
Q

most common inheritable cause of a stroke

A

activated protein C resistance (factor 5 Leiden)

174
Q

extensive collateral vessels from prior occlusion around the circle of willis on cerebral angiography of child with sickle cell

A

moyamoya

175
Q

what arterial distribution do most neonatal cerebral infarctions occur

A

left middle cerebral artery

176
Q

most common location for skull fractures in kids

A

parietal

177
Q

EEG pattern with generalized bilateral synchronous sharp wave and slow wave complexes occurring in a repetitive fashion in long runs at ~2 Hz

A

Lennox gastaut

178
Q

diagnosis with history of focal unremitting seizures on L side of the body with progressive hemiparesis on that side and atrophy of the R hemisphere on MRI

A

Rasmussen syndrome

179
Q

type of migraine with vertigo, syncope, dysarthria, visual alterations and LOC

A

basilar artery migraine

180
Q

diagnosis with worsening muscle weakness exacerbated by repetitive muscle use an ocular muscle weakness, ptosis

A

juvenile myasthenia gravis

181
Q

diagnosis with optic neuritis, oculomotor disturbances, incoordination of upper extremities, sensory deficits, CSF with IgG and oligoclonal bands, MRI with areas of active demyelination and evidence of prior demyelination

A

multiple sclerosis

182
Q

SMA type 1 is carried on what gene/chromosome

A

SMN1 gene on chromosome 5q13

183
Q

how to confirm diagnosis of Duchenne muscular dystrophy

A

identify mutation on the DMD gene (if negative and high suspicion then muscle biopsy)

184
Q

diagnosis in newborn of mom with a neurologic disease that at birth has hypotonia, weak cry, difficulty feeding, facial weakness, ptosis and respiratory distress

A

neonatal myasthenia gravis from transplacental transmission of maternal acetylcholine receptor antibodies (AChR-Ab) - resolves in 2 to 6 weeks

185
Q

treatment for juvenile myasthenia gravis

A

oral anticholinesterase meds (pyridostigmine)
immunosuppression
thymectomy
plasmapheresis or IVIG