Neuro Flashcards

1
Q

Breath holding spells

  • age
  • 2 types
  • assoc w?
  • usually resolve by what age
  • mgmt
A

6-18 mos old

• Cyanotic – “the angry infant” – apnea, cyanosis
after agitation, crying
• Pallid – “the injured infant” – limp & pallor after an injury

assoc w Iron deficiency anemia

5 years old

reassure and consider iron supplement

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2
Q

Infantile Spasms

  • age
  • description
A

peak onset 4-7 mo, almost always < 1yo

last 1 sec
occur in CLUSTERS
neck, trunk flexion
arm extension
symmetric, synchronous

association:
TS (also HIE, stroke, T21, etc)

long term: neurodevelop problems, and seizure d/o

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3
Q

Infantile Spasms

  • Dx
  • Tx
  • what to tx if TS
A

EEG: hypsarrhythmia

Tx:
vigabatrin
high dose prednisone
ACTH

TS: tx w vigabatrin

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4
Q

West Syndrome

A

Infantile spasms
Hypsarrhythmia
Mental Retardation

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5
Q

Sandifer Syndrome

- key points

A
  • Abnormal movements (axial stiffening) due to GERD

* Usually occur with or after feeds in a “spitty” baby

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6
Q

Benign Myoclonus of Infancy

  • presentation
  • exam/EEG
  • tx
A

“shudder attacks”

presents at 4-6mo (<2yo)

sudden brief symmetrical axial flexor spasms of trunk &head lasting 1-2 sec OR “vibratory” flexion of neck
- May be provoked by excitement / fear

Normal exam
Normal EEG

No tx
Spontaneous remission by 5 years of life

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7
Q

Benign sleep myoclonus of infancy

A

Birth - 3mo

Discrete limb jerks when asleep

Always resolve upon awakening
Never when awake

N exam, N eeg

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8
Q

Infantile Masturbation

A

F>M

  • Pelvic rocking.
  • Pelvic pressure
  • Adduction of legs
  • Flushing, diaphoresis (autonomic phenomena)
  • May be difficult to distract out of episode
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9
Q

Childhood Absence Epilepsy

  • char
  • eeg
  • tx
  • prognosis
A

onset 5-7 years peak (4-10)
Blank stare 5-30 sec
interrupts what they’re doing
up to 100x/day

eeg: 3Hz spike and wave

Tx:
Ethosuxamide, VPA (or lamotrigine)
75% complete remission by adolescence

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10
Q

Benign rolandic epilepsy

A

7-10 yrs old (range 1-14 yrs)

  • Nocturnal focal seizures of Rolandic area (face, tongue)
  • May awaken with inability to speak (“sleep walker”)
  • May have rhythmic facial twitching
  • May spread to be generalized tonic-clonic seizure

eeg: Centrotemporal spikes

No Treatment usually
(if need, levetiracetam, carbamazepine)
Most outgrow

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11
Q

What is ethosuccimide used for

A

absense

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12
Q

Juvenile myoclonic epilepsy

  • age
  • presentation
  • EEG
  • Tx
  • prgonosis
A

late childhood/early adolescence

Early AM myoclonus (clumsiness)

EEG: gen. fast spiking waves

Tx: VPA, lamotrigine, keppra

Prognosis: lifelong epilepsy req’ing tx

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13
Q

Anticonvulsants used for primary generalized epilepsy

A
  • Valproic acid (VPA)
  • Lamotrigine (LTG)
  • Levetiracetam (LEV)
  • Topiramate (TPM)
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14
Q

Anticonvulsants used for secondarily generalized epilepsy (FOCAL)

A
  • Levetiracetam (Keppra)
  • Carbamazepine (tegretol) / oxcarbamazpine

LEAVE the CARBS

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15
Q

VPA

  • who should not get
  • S/E
A

< 2yo

wt gain
hair loss
tremor
PCOS
incr LFTs
pancreatitis
thrombocytopenia
hyperammonemia
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16
Q

Status epilepticus

- mgmt

A

ABC’s, gluc/labs, IV access

Lorazepam 0.1mg/kg x2

Fosphenytoin 20mg/kg or Phenobarbital 20mg/kg

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17
Q

Febrile Seizure

- simple vs complex

A
SIMPLE
generalized
<15 min
no recurrence w/I 24 hr
no post ictal 
COMPLEX
focal onset
>15 min
2 or more in 24 hr
\+ post ictal
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18
Q

Increased risk of recurrence of febrile seizure

A

young age
complex
FHx
Sz w low fever

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19
Q

Febrile Seizure

  • recurrence risk
  • risk of epilepsy
A
  • recurrence risk: 1/3

- risk of epilepsy: 2.4% (compared to 1%)

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20
Q

Antiepileptic meds - SJS

A

Carbemazepine

Phenytoin

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21
Q

Children < 2 - anticonvulsants

A
  • Phenobarbitol
  • Levetiracetam
  • Topiramate
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22
Q

Migraine

  • how many
  • criteria
A

At least 5 attacks
1-72h

SULTANS
- 2 or more of:
• Severity: Moderate-to-severe pain (miss school)
• UniLateral or bilateral (frontotemporal,
not occipital)
• Throbbing/Pulsing quality
• Aggravated by activity (climbing stairs)

  • at least 1 of:
    • Nausea +/- vomiting
    • Sensitivities: Photophobia and phonophobia

Cannot be explained by another disorder

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23
Q

Migraine

treatment

A

ABORTIVE

  • Ibuprofen
  • Triptans
  • Acetaminophen

PREVENTATIVE

  • lifestyle (food triggers, sleep, hydration, exercise, stress, avoid caffeine)
  • Med: amitriptyline, topiramate, flunarazine, sandomigraine
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24
Q

Thunderclap headache

A

red flag for sentinel bleed

- aneurysm

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25
Q

Occipital location

A

Chiari 1 malformation

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26
Q

Red flags for h/a

A
Sudden (thunderclap)
Occipital
Pituitary sx (growth, bedwet, vision loss)
Awake from sleep
Focal deficit on p/w
Head injury
Coagulopathy
Risk of thrombosis
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27
Q

Medication overuse

A

Basically:
15 days/week of headache and of med use
for 3 months

  • Daily (or near-daily) headache
  • Present upon awakening
  • Better (or relieved) with medication
  • Recurs later in day
  • Headaches >15 days per month (often daily)

Regular use of:
• Ibuprofen or acetaminophen >15x/mos over >3 mos
• Triptans >10x/mos over >3 mos

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28
Q

Tension headache

  • char
  • tx
A
bilateral
pressing/tightening
mild-mod 
not aggravated by physical activity
no N/V

tx: acetaminophen

29
Q

Benign intracranial hypertensions: Meds

A

OCP
minocycline
tetracycline
retinoid acid

30
Q

Central hypotonia

A

ex: T21, PWS

seizures more likely
normal reflexes
weak but does have anti gravity reflexes

31
Q

absent reflexes - type

A

peripheral hypotonia
SMA (motor neuron)
Charcot (nerve)
LATE: muscular dystrophy (muscle problem)

32
Q

Muscle Weakness

  • prox
  • distal
A

Prox:
muscular dystrophy
Duchenne/becker
SMA

Distal:
GBS (polyneuropathy)
myotonic dytrophy

33
Q

SMA

  • type
  • characteristics
  • diagnosis
A

motor neuron disorder

ABSENT REFLEXES
Pros weakness/wasting
Face spared

Genetic testing - SMN1 deletion

34
Q

Charcot Marie Tooth

A

Nerve disorder
Areflexia
distal weakness
sensory problems

EMG
Genetic testing

Infant
• Delayed gross motor milestones,
• Tight heel cords: “toe-walker”, clumsy gait 
•Hypotonia. Weakness
• Self-mutilation

Children
• Abnormal gait & clumsiness.
• Foot deformities: pes cavus, hammertoes
• Weakness + foot drop

35
Q

X- linked adrenoleukodystrophy

  • 4 features
  • dx
A

Xlinked
perioxisomal d/o

male
ataxia
ADHD features/behaviour
Addison

dx: increased VLCFA

36
Q

Duchenne’s Musclular Dystrophy

A

xlinked
Progressive destruction of muscle

presents 3-4 yo
Toe walking
Waddling
Difficulty rising from floor/up stairs

Gower’s sign
Gastronemius psuedohypertrophy

Elevated CK

Dx: genetic

37
Q

Guillain Barre Syndrome

  • cause
  • characteristics
  • invest
  • mgmt
A

acute inflammation demylenating polyradiculopathy

caused by:
Campylobacter jejuni
Mycoplasma pneumoniae

Areflexia
Flacidity
Symmetric ascending weakness
Autonomic dysfunction

CSF: increased Pn w/o pleocytosis

MGMT
admit, support
IVIG

38
Q

Incontinenti pigmenti

A

seizures & vesicular lesions in a dermatomal distribution

39
Q

Neurofibromatosis
- inheritance
-

A

AD

2/7:

  • first degree relative with NF1
  • 6+ cafe au lait
    • > 5mm (prepubertal)
    • > 15mm (postpubertal)
  • axillary, inguinal or neck freckling
  • 2+ neurofibromas or 1 plexiform (usually during puberty or pregnancy; plexiform at birth, purple rubbery around face)
  • 2+ Lisch nodules - hamartomas of the iris - increase incidence with age
  • optic glioma - benign, usually asymptomatic - if unilateral get afferent pupil defect (dilates with light)
  • osseous lesions - sphenoid dysplasia, thinning of long bones, scoliosis
40
Q

Baby w facial droop

A

Able to wrinkle forehead:
CONGENITAL ABSENCE OF THE DEPRESSOR ANGULARIS ORIS MUSCLE

Unable to wrinkle forehead
Bells Palsy

41
Q

Brachial Plexus Injury

A

C5-T1

RF:
Birth trauma
Shoulder dystocia
LGA
GDM
Instrumentation

75% resolve by 1 month
if not. refer

42
Q

Post Infectious Acute Cerebellar Ataxia

A

gradual onset of gait disturbance, nystagmus, and slurred speech

> 25% varicella

43
Q

Rett Syndrome

A
  • repetitive hand-wringing movements
  • loss of purposeful and spontaneous use of the hands
  • Autistic behavior
  • Generalized tonic-clonic convulsions
  • Feeding disorders and poor weight gain are common.
  • microcephaly: early deceleration of head growth, followed by deceleration of weight and height measurements
44
Q

diplopia, head tilt and ataxia

A

cerebellar astrocytoma

45
Q

spastic diplegia

A

lower limbs

assoc w PVL (<32wk)

46
Q

Erb’s palsy

  • what nerves
  • presentation
A

C5, C6
shoulder dystocia causing stretching of brachial plexus.

Waiter’s tip hand (shoulder internally rotated, arm adducted, elbow extended, hand pronated).
Usually gets better.

47
Q

Klumpke’s palsy

A

C8, T1

Deficits of small muscles of hand, presents as claw hand, usually permanent deficits.

48
Q

Duchenne Muscular Dystrophy

AM h/a

A

nocturnal hypoventilation
resp acidosis
overnight BiPAP

49
Q

persistence of the asymmetric tonic neck reflex at 9 months of age

A

Spastic cerebral palsy

50
Q

tuberous sclerosis - inheritiance

A

Autosomal dominant

51
Q

Contraindications to LP

A
  • suspected mass lesion of the brain
  • suspected mass lesion of the spinal cord;
  • symptoms and signs of impending cerebral herniation in a child with probable meningitis;
  • critical illness (on rare occasions);
  • skin infection at the site of the lumbar puncture
  • thrombocytopenia with a platelet count of < 20 × 10 9 /-
  • coagulopathy
  • parents don’t consent
52
Q

Gradenigo syndrome

A

otitis media, facial pain, and abducens palsy

ceftriaxone and metronidazole

53
Q

GBS - investigations

- treatment

A
  • LP – high CSF protein, no other abnormalities (normal WBCs)
  • MRI head and spine – contrast enhancement of CN roots, spinal nerve roots, cauda equina
  • Nerve conduction studies – motor conduction block, slowing (or decreased amplitude) of conduction, temporal dispersion, prolonged latencies
  • Serum auto-Ab – anti-GQ1b
  • IVIg
  • Plasmapheresis
  • Supportive – esp. respiratory, assisted ventilation
    (not steroids)
54
Q

Causes of regression?

A

SPHINGOLIPIDOSES
- Niemann-Pick Disease: Cognitive regression, hepatosplenomegaly, jaundice, seizures
- Gaucher Disease: Hepatosplenomegaly, cytopenia, spasticity, hyperextension, extraocular palsies, trismus, difficulty swallowing
- GM1 Gangliosidoses: Infantile form: early feeding difficulties, GDD, sz, coarse facial features, hepatosplenomegaly, cherry red spot
Juvenile form: incoordination, weakness, language regression; later, seizures, spasticity, blindness
- GM2 Gangliosidoses
- Tay-Sachs Disease—progressive weakness, marked startle reaction, blindness, convulsions, spasticity, and cherry red spot
- Sandhoff Disease—phenotype similar to Tay-Sachs

X-LINKED ADRENOLEUKODYSTROPHY
- Classic Adrenoleukodystrophy: Academic difficulties, behavioral disturbances, hypoadrenalism, seizures, spasticity, ataxia, and swallowing difficulties.

MUCOPOLYSACCHARIDOSES (MPS): Short stature, kyphoscoliosis, coarse facies, hepatosplenomegaly, cardiovascular abnormalities, and corneal clouding
TYPES: Hurler (MPS type I-H), Hunter (MPS type II), Sanfilippo Disease (MPS type III), Sly Syndrome (MPS type VII)

MITOCHONDRIAL DISORDERS
- MELAS: Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes

SUBACUTE NECROTIZING ENCEPHALOMYELOPATHY (LEIGH DISEASE): Hypotonia, feeding difficulties, respiratory irregularity, weakness of extraocular movements, and ataxia
Test: depends on clinical phenotype; blood and CSF lactate and pyruvate are elevated

RETT SYNDROME
Loss of purposeful hand movements and communication skills, social withdrawal, gait apraxia, seizures, spasticity, and kyphoscoliosis
Test: MECP2 gene testing

WILSON DISEASE
Inborn error of copper metabolism resulting in signs of cerebellar and basal ganglia dysfunction
Tests: serum ceruloplasmin levels, urinary copper excretion

55
Q

complications of spina bifida

A

1- Hydrocephalus (77-95%): does appear to have an association with level of lesion. Treatment is placement of a ventricular shunt or endoscopic third ventriculostomy

2- Hindbrain herniation or the Chiari type II malformation (80-90%) = caudal displacement of the cerebellum, pons, and medulla and elongation of the fourth ventricle. *symptomatic (from brainstem herniation/compression) in approximately 20%

3- Risk of tethered cord syndrome. After shunt malfunction, this is the second most common cause for neurologic decline. Clinical manifestations include any change in gait or bowel or bladder function, increasing scoliosis, back pain, or orthopedic changes. Surgical detethering procedures are indicated in those with neurologic decline but the success rate is variable.

4- Osteoporosis, scoliosis, hip dislocation, club foot
5- Neurogenic bladder and bowel
6- Learning disabilities but typically have average IQ range

56
Q

reason why diazepam is not commonly used as an anticonvulsant in neonates

A

Decreased liver metabolism

57
Q

EEG:

centrotemporal

3Hz

A

centrotemporal: benign rolandic

3Hz: absense

58
Q

Most common cause of seizures in adolesents

A

Genetic - juvenile myoclonic epilepsy

59
Q

Sturge Weber

  • features
  • Dx
  • Progonisis
A
Port wine stain - V1 distribution 
Seizures
Glaucoma (need to screen for)
Stroke like episodes
Developmental delay
Complications:
S- seizures
S- stroke like episodes
H- hemiparesis
H- hemianopsia
H- headache
D- Developmental disabilities
G- Glaucoma

Diagnosis:
MRI with contrast showing leptomeningeal angioma; white matter abnormalities are common; often atrophy is noted ipsilateral to the leptomeningeal angiomatosis

Neurodevelopment tends to appear normal in first year of life, then intellectual disability or severe learning disabilities are present in at least 50% in later childhood

60
Q

Teratogen effect

Carbemazapine
Phenytoin
VPA
Topiramate

A

Carbemazapine: neural tube, neurodevel delay

Phenytoin: congenital anomaly, IUGR, neuroblastoma, bleeding (VIT K)

VPA: neural tube, cardiac anomalies, facial anomalies, limb defects

Topiramate: cleft lip/palate

61
Q

Signs of CP

A
Early hand preference
Delayed fine/gross motor development
Spasticity
Tip toe walking
Ankle clonus
Hyperreflexia
Flexion contractures
62
Q

Posterior fossa tumours

A

headache
nausea, and vomiting
papilledema

63
Q

Varicella - neuro sx

A

Acute cerebellar ataxia

64
Q

acute cerebellar ataxia

  • age
  • presentation
  • CSF
  • prognosis
A

1-3 yr of age
diagnosis of exclusion
follows a viral illness (varicella , coxsackievirus, or echovirus) by 2-3 w

autoimmune response to the viral agent affecting the cerebellum. 
typically sudden onset
\+ Vomiting 
NO fever 
NO nuchal rigidity
\+ Horizontal nystagmus 
dysarthria 

CSF:
initially N or mild lymphocytic pleocytosis (10-30/mm 3)
Later: moderately elevated protein

Tx: Steroids or IVIG

Prognosis:
Ataxia begins to improve in a few weeks but may persist for as long as 3 mo.

The prognosis for complete recovery is excellent.
A small number of patients have long-term sequelae, including behavioral and speech disorders, as well as ataxia and incoordination.

65
Q
IVH - chance of neurological sequelae
Grade 1
Grade 2
Grade 3
Grade 4
A

Grade 1 - 15%
Grade 2 - 25%
Grade 3 - 50%
Grade 4 - 75%

66
Q

Signs of increased ICP

A
Change in LOC
Papilledema
Pupillary Changes
Impaired eye movements
Posturing - decerebrate or decorticate
Decreased motor fn 
H/A
Seizure
Change in vitals
- Cushing's triad
- altered resp pattern
Vomiting
Changes in speech
Infants
Bulging fontanelle
Cranial suture separation
Increased HC
High pitched cry
67
Q

Duchenne muscular dystrophy

- organs involved

A
  • Respiratory system: nocturnal hypoventilation; ineffective cough due to weak diaphragm and thoracic muscles
  • Cardiac system: dilated cardiomyopathy
  • Brain: Intellectual disability
  • Head and neck: swallowing dysfunction
  • GI: constipation
68
Q

Botulism

A

ophthalmoplagia
limb weakness (descending flaccid paralysis)
facial and bulbar weakness

69
Q

BRUE

A

Criteria (dx of exclusion)
- <1 year old
- Sudden, brief, and now resolved
Aka returned to baseline

  • Must include at least 1 or more of
    Cyanosis or pallor
    Absent, decreased, or irregular breathing
    Change in muscle tone (hyper or hypotonia )
    Altered level of responsiveness