Neuro Flashcards
Breath holding spells
- age
- 2 types
- assoc w?
- usually resolve by what age
- mgmt
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
Infantile Spasms
- age
- description
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
Infantile Spasms
- Dx
- Tx
- what to tx if TS
EEG: hypsarrhythmia
Tx:
vigabatrin
high dose prednisone
ACTH
TS: tx w vigabatrin
West Syndrome
Infantile spasms
Hypsarrhythmia
Mental Retardation
Sandifer Syndrome
- key points
- Abnormal movements (axial stiffening) due to GERD
* Usually occur with or after feeds in a “spitty” baby
Benign Myoclonus of Infancy
- presentation
- exam/EEG
- tx
“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
Benign sleep myoclonus of infancy
Birth - 3mo
Discrete limb jerks when asleep
Always resolve upon awakening
Never when awake
N exam, N eeg
Infantile Masturbation
F>M
- Pelvic rocking.
- Pelvic pressure
- Adduction of legs
- Flushing, diaphoresis (autonomic phenomena)
- May be difficult to distract out of episode
Childhood Absence Epilepsy
- char
- eeg
- tx
- prognosis
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
Benign rolandic epilepsy
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
What is ethosuccimide used for
absense
Juvenile myoclonic epilepsy
- age
- presentation
- EEG
- Tx
- prgonosis
late childhood/early adolescence
Early AM myoclonus (clumsiness)
EEG: gen. fast spiking waves
Tx: VPA, lamotrigine, keppra
Prognosis: lifelong epilepsy req’ing tx
Anticonvulsants used for primary generalized epilepsy
- Valproic acid (VPA)
- Lamotrigine (LTG)
- Levetiracetam (LEV)
- Topiramate (TPM)
Anticonvulsants used for secondarily generalized epilepsy (FOCAL)
- Levetiracetam (Keppra)
- Carbamazepine (tegretol) / oxcarbamazpine
LEAVE the CARBS
VPA
- who should not get
- S/E
< 2yo
wt gain hair loss tremor PCOS incr LFTs pancreatitis thrombocytopenia hyperammonemia
Status epilepticus
- mgmt
ABC’s, gluc/labs, IV access
Lorazepam 0.1mg/kg x2
Fosphenytoin 20mg/kg or Phenobarbital 20mg/kg
Febrile Seizure
- simple vs complex
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
Increased risk of recurrence of febrile seizure
young age
complex
FHx
Sz w low fever
Febrile Seizure
- recurrence risk
- risk of epilepsy
- recurrence risk: 1/3
- risk of epilepsy: 2.4% (compared to 1%)
Antiepileptic meds - SJS
Carbemazepine
Phenytoin
Children < 2 - anticonvulsants
- Phenobarbitol
- Levetiracetam
- Topiramate
Migraine
- how many
- criteria
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
Migraine
treatment
ABORTIVE
- Ibuprofen
- Triptans
- Acetaminophen
PREVENTATIVE
- lifestyle (food triggers, sleep, hydration, exercise, stress, avoid caffeine)
- Med: amitriptyline, topiramate, flunarazine, sandomigraine
Thunderclap headache
red flag for sentinel bleed
- aneurysm
Occipital location
Chiari 1 malformation
Red flags for h/a
Sudden (thunderclap) Occipital Pituitary sx (growth, bedwet, vision loss) Awake from sleep Focal deficit on p/w Head injury Coagulopathy Risk of thrombosis
Medication overuse
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
Tension headache
- char
- tx
bilateral pressing/tightening mild-mod not aggravated by physical activity no N/V
tx: acetaminophen
Benign intracranial hypertensions: Meds
OCP
minocycline
tetracycline
retinoid acid
Central hypotonia
ex: T21, PWS
seizures more likely
normal reflexes
weak but does have anti gravity reflexes
absent reflexes - type
peripheral hypotonia
SMA (motor neuron)
Charcot (nerve)
LATE: muscular dystrophy (muscle problem)
Muscle Weakness
- prox
- distal
Prox:
muscular dystrophy
Duchenne/becker
SMA
Distal:
GBS (polyneuropathy)
myotonic dytrophy
SMA
- type
- characteristics
- diagnosis
motor neuron disorder
ABSENT REFLEXES
Pros weakness/wasting
Face spared
Genetic testing - SMN1 deletion
Charcot Marie Tooth
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
X- linked adrenoleukodystrophy
- 4 features
- dx
Xlinked
perioxisomal d/o
male
ataxia
ADHD features/behaviour
Addison
dx: increased VLCFA
Duchenne’s Musclular Dystrophy
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
Guillain Barre Syndrome
- cause
- characteristics
- invest
- mgmt
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
Incontinenti pigmenti
seizures & vesicular lesions in a dermatomal distribution
Neurofibromatosis
- inheritance
-
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
Baby w facial droop
Able to wrinkle forehead:
CONGENITAL ABSENCE OF THE DEPRESSOR ANGULARIS ORIS MUSCLE
Unable to wrinkle forehead
Bells Palsy
Brachial Plexus Injury
C5-T1
RF: Birth trauma Shoulder dystocia LGA GDM Instrumentation
75% resolve by 1 month
if not. refer
Post Infectious Acute Cerebellar Ataxia
gradual onset of gait disturbance, nystagmus, and slurred speech
> 25% varicella
Rett Syndrome
- 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
diplopia, head tilt and ataxia
cerebellar astrocytoma
spastic diplegia
lower limbs
assoc w PVL (<32wk)
Erb’s palsy
- what nerves
- presentation
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.
Klumpke’s palsy
C8, T1
Deficits of small muscles of hand, presents as claw hand, usually permanent deficits.
Duchenne Muscular Dystrophy
AM h/a
nocturnal hypoventilation
resp acidosis
overnight BiPAP
persistence of the asymmetric tonic neck reflex at 9 months of age
Spastic cerebral palsy
tuberous sclerosis - inheritiance
Autosomal dominant
Contraindications to LP
- 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
Gradenigo syndrome
otitis media, facial pain, and abducens palsy
ceftriaxone and metronidazole
GBS - investigations
- treatment
- 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)
Causes of regression?
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
complications of spina bifida
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
reason why diazepam is not commonly used as an anticonvulsant in neonates
Decreased liver metabolism
EEG:
centrotemporal
3Hz
centrotemporal: benign rolandic
3Hz: absense
Most common cause of seizures in adolesents
Genetic - juvenile myoclonic epilepsy
Sturge Weber
- features
- Dx
- Progonisis
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
Teratogen effect
Carbemazapine
Phenytoin
VPA
Topiramate
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
Signs of CP
Early hand preference Delayed fine/gross motor development Spasticity Tip toe walking Ankle clonus Hyperreflexia Flexion contractures
Posterior fossa tumours
headache
nausea, and vomiting
papilledema
Varicella - neuro sx
Acute cerebellar ataxia
acute cerebellar ataxia
- age
- presentation
- CSF
- prognosis
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.
IVH - chance of neurological sequelae Grade 1 Grade 2 Grade 3 Grade 4
Grade 1 - 15%
Grade 2 - 25%
Grade 3 - 50%
Grade 4 - 75%
Signs of increased ICP
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
Duchenne muscular dystrophy
- organs involved
- 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
Botulism
ophthalmoplagia
limb weakness (descending flaccid paralysis)
facial and bulbar weakness
BRUE
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
