Neuro Flashcards
What are the 3 different spinal cord tracts and how to test them?
Dorsal (posterior) column (sensory)
○ Proprioception, vibration, light touch
○ Ipsilateral findings
○ Testing: position of fingers and toes, vibration using tuning fork
Spinothalamic (sensory)
○ Pain, temperature, touch
○ Injuries show as contralateral findings for pain and temperature
○ Testing: pinprick sensation, temperature
Corticospinal tract (motor)
○ Controls motor function on same side
○ Ipsilateral findings
Testing: abnormal motor movements on same side of lesion
Lower motor neuron findings
Bulk: atrophy, fasciculations (muscle fibers twitching)
Tone: flaccid
Pattern: root/plexus/nerve distribution
Reflexes: absent/diminished, toes down
Root, nerve
Upper motor neuron findings
Bulk: normal
Tone: spastic (tight muscles)
Pattern: extensors affected more in arms, flexors more in legs
Reflexes: brisk (intense), up-going toes (Babinski reflex)
Cortex to spinal cord
opening pressure 90%ile ULN and 10%ile LLN
28cm H2O high
11.5 cm low
contraindications to LP
suspected mass lesion (esp posterior fossa)
suspected spinal cord lesion
signs of impending herniation
critical illness
skin infection over the site
Plt <20
*routine head imaging not needed unless signs of optic disc edema or focal signs suggesting mass lesion
Normal CSF
up to 5 WBC (up to 15 in newborns)
polymorphonuclear cells are always abnormal in a child (may have 1-2 in neonate)
xanthochromia suggests subarachnoid hemorrhage (can occur in hyperbilirubinemia, carotenaemia, or high CSF protein)
early signs of stroke on CT <24hr
CT isnt great for acute infarcts due to changes not being apparent in first 24 hr
subtle signs:
- sulcal effacement
- blurring of gray-white matter junction
- hyperdense MCA sign
brachial plexus injuries
C3-C4
-C3,C4,C5 keeps the diaphragm alive
C5-C6
-Biceps
-Erb’s palsy
-Pronate, retained grip strength
C7-C8
-Klumpke palsy
-Claw hand (can’t properly flex or abduct the fingers)
Ramsay hunt syndrome
idiopathic bell’s palsy (herpes zoster)
- steroids + acyclovir
Upper limb myotome testing
Upper memory tool - start at 5 - shoulders (5), bicep curl (6), tricep extension (7), fist (8), fingers spread (T1)
Upper limb nerve root testing
Nerve testing - circle is median, thumbs up is rad, fingers splayed ulnar
An infant presents with roving eye movements, jerking of the extremities, and ataxia. What is the cause?
opsoclonus myoclonus
associated with neuroblastoma
Workup with Urine HMA/VMA or MIBG Scan
Cerebellitis
self limiting process usually following viral infection (ex. varicella, coxsackie, echovirus)
Gait disturbance
Associated with nystagmus, slurred speech, vomiting, irritability, dysarthria, headache, ataxia, dysdiadochokinesia
Differentiate from encephalitis by: no fever, no nuchal rigidity, no seizures, normal WBC and protein on CSF
Guillain Barre treatment
IVIG
Guillain Barre CSF finding
cytoalbuminologic dissociation
- increased protein
- normal to low WBC
early hand preference
typically a missed stroke causing spastic hemiplegia
dyskinetic CP
dystonia, chorea, athetosis
may not present until 2 yrs
usually associated with perinatal distress of kernicterus
DMD inheritance
x linked recessive (out of frame deletion)
You are seeing a 5-year-old boy who is having difficulty walking now after he previously walked at 12 months. His development has otherwise been normal. You notice that he has very well-defined calves. What test would be most in keeping with your suspected diagnosis?
duchenne muscular dystrophy
do a CK
X linked recessive
SeLECTS/BECTS epilepsy treatment
Focal seizures with motor symptoms involving the face - numbness, twitching, hypersalivation, guttural vocalizations, speech arrest
Most occur at night or on awakening
No impairment of awareness
Can progress to GTC
NO treatment needed
remits by age 15
Teenager wakes up and has messed up face and drooling and then it resolves
-Dx? Tx?
SELECTS seizures/Benign Rolandic Epilepsy
NO TREATMENT NEEDED -> unless impairing sleep and school performance then consider carbamazepine
Self resolves by 15yrs old
child presenting with dropping objects in the morning
juvenile myoclonic epilepsy
- treat with VPA > lamotrigine > keppra
- life long treatment
Side effect of ethosuximide
agranulocytosis
side effect phenytoin
gingival hyperplasia
ataxia
SJS
workup indications for complex febrile seizures
Consider LP, EEG, Neuroimaging if:
- Questionable hx of fever
- Focal seizure
- Hx of devel delay
- Abnormal neuro exam (dysmorphic, focal neuro, neurocutaneous findings)
Newborn baby born with hypotonia. History of decreased fetal movements. Normal facial movements. Alert. Weak extremities and low tone. Absent reflexes. What is the most likely diagnosis?
SMA
SMA inheritance
autosomal recessive
Best test for myasthenia gravis
EMG (decremental response to repetitive stimulation)
tuberous sclerosis inheritance
autosomal dominant
4yo male with multiple hypomelanotic macules, rough raised lesion with orange peel consistency in the lumbar area, 3 cafe’ au lait spots and retinal hamartoma. What are they most at risk for?
cardiac rhabdomyoma
in tuberous sclerosis
Major features of tuberous sclerosis
ASHLEAF (for major criteria)
- Ashleaf spots (hypopigmentation)
- Shagreen patches (orange peel consistency, usually in the lumbosacral region)
- Heart rhabdomyosarcoma
- Lung hamartomas
- Epilepsy from cortical tubers/dysplasias
- Angiomyolipoma in kidneys
- Facial angiofibromas
+ Multiple retinal nodular hamartomas
+ Astrocytomas
tuberous sclerosis screening and surveillance
brain MR q1-3yr
optho q1 yr
echo q 1-3 yr
renal imaging
adrenoleukodystrophy diagnosis
serum very long chain fatty acids
x linked disorder
5 year old male who suddenly stops talking. Previously otherwise well. He recently had a generalized seizure and EEG during sleep shows epiletiform activity. What is the most likely diagnosis?
landau kleffner
- normal language until 3-7yrs
diagnosis tourettes
2 motor tics and 1 vocal tic for 1 year
anti NMDA encephalitis
- often behavioural for months (anxiety, mood, sleep disturbance, psychosis)
- later develop seizures and dystonia and autonomic dysfxn (htn, tachycardia)
- dx: CSF antibodies
- can be triggered by HSV encephalitis or ovarian teratomas
anti GABA encephalitis
- presents with refractory seizures
- MRI: Multifocal cortical subcortical FLAIR/T2 hyperintensities
Acute Disseminated Encephalomyelitis (ADEM) encephalitis
- anti mog
- post infectious, rapidly progressive encephalitis
- seizures, motor deficits, ataxia, visual impairments
MR: large hazy abnormalities on T2/flair
NMO encephalitis
- anti-aquaporin
- affects optic nerve and spinal cord
- high risk relapse
- immunotherapy long term
infantile spasm treatment
steroids or vigabatrin
4 year old girl with 3 GTC seizures in past week. Several days of emotional lability and confusion. In ED, mild tachycardia and hypertension. Abnormal (writhing) mouth/facial movements.
Which diagnostic test will best determine the diagnosis?
CSF auto antibodies for autoimmune encephalitis
17 yo girl with 8 month history of amenorrhea and headaches. Intermittent bothersome galactorrhea. High prolactin on bloodwork. MRI showed adenoma 5mm x7mm. What is the best treatment option?
dopamine agonist
Teenage kid with new behavioral concerns, anxiety, depression. On examination has hepatomegaly and intentional tremor. Bloodwork shows DAT negative hemolytic anemia. What will you recommend?
Wilson disease
restrict dietary intake of copper (liver, shellfish, nuts, chocolate)
keiser flischer rings
wilsons disease
wilsons disease presentation
- liver disease with high liver enzymes, hepatomegaly
- psychiatric disturbance
- motor symptoms (ataxia, tremor, seizures)
- KF rings
- renal tubular dysfunction
- DAT negative hemolytic anemia
low risk BRUE criteria
Age > 60 days
If premature, gestational age >/= 32 weeks and CGA >/= 45 wks
First episode of BRUE
Duration < 1 min
No CPR required by trained medical provider
No concerning features on history or physical
Rett syndrome
x linked dominant (but boys die so just in girls)
MECP2
regression, acquired microcephaly, ataxia, wringing hands, autistic behaviours
In patients with TS, MRI brains are required every ____yrs to monitor for ______
1-3 years
giant cell astrocytomas
Second line medications in status epilepticus?
Fospheny OR Pheny 20mg/kg (do not give both, do not use if concern for overdose)
Keppra 60mg/kg (good choice if concerned about resp depression)
VPA 30mg/kg (do not use if devel delay/ <2)
Phenobarb 20mg/kg (use in overdose scenarios or in kids <6mo, can cause resp depression, hypotension, sedation)
After giving 1 second line med, if pt still seizing in 5 minutes, give a second med
Features of simple vs complicated febrile seizures
Simple = generalized, <15 mins, no reoccurance within 24hrs, no focal neuro deficits on exam
Complex = focal, >15 mins, >2 in 24hrs, post ictal focal deficits
RF for reoccurance of febrile seizure?
- family history of febrile seizure
- family history of epilepsy
- seized at a lower temp
- complex
- age <1yr
EEG pattern for Absence Sz
3Hz spike and wave
Tx for Absence Sz
Ethosuximide
Most will outgrow by adolesence
6 day old infant, now having 2-3 min tonic clonic seizures occurring 20-30 times per day with associated apneas. Normal pregnancy, uncomplicated delivery, normal neurological exam. Dad had seizures as a baby. Dx and Tx?
Benign familial neonatal epilepsy (SeLFNIE)
- Autosomal dominant
- resolves by 6 weeks- 1 year
- will respond to Na channel blockers (carbamazepine, phenobarb)
EEG pattern in Infantile spasms
Hypsarrythmia (high amplitide waves on background of irregular spikes)
Clinical presentation of Infantile spasms
Flextion at the waist, extension of the limbs, occurs in CLUSTERS and can be associated with sleep transitions
Sandifer syndrome
Spasms of neck extension and back arching, spares the limbs, occurs around feeding.
Associated with GERD and can mimic Infantile spasms
EEG pattern in SELECTS/Benign Rolandic Epilepsy
Centrotemporal spikes
Dravet Syndrome
Complex, clustering febrile seizures that progress to occurring without fever and cause devel delay
Side effects of carbamazepine?
SJS
Agranulocytosis
Hepatitis
Rash
SIADH
Ataxia
Side effects of Topiramate?
Kidney stones
Cognitive slowing
Weight loss
Metabolic acidosis
Aura with deja vu, or stomach upset followed by fixed stare, fumbling with fingers, lip smacking
Temporal lobe epilepsy
Abortive ED management of migraine?
IVF bolus
Acetaminophen +NSAID
Metoclopramide
If still in pain at 1hr-> consult neuro for ?VPA
Inheritance pattern of DMD?
X linked recessive (duplication deletion on chromosome 21)
Inheritance patter of myotonic dystrophy?
AD
What treatment prolongs ambulation time and improves respiratory status in DMD?
Steroids
Clinical presentation of myotonic dystrophy?
- Bilateral non fatigable ptosis
- Tented mouth
- Narrow high arched palate
- Inverted V lip
- Distal muscle wasting first and then proximal
- Reflexes preserved
- Cognitive impairment
Clinical presentation of myasthenia gravis:
- Fatiguable weakness and ptosis
- Can also have difficulty swallowing and slurred speech
- DTRs diminished but not lost
Clinical presentation of botulinum toxin?
- Constipation followed by bulbar weakness and descending acute flaccid paralysis
- Fatiguability
Infections associated with Guillain Barre?
Campylobacter
Mycoplasma pneumoniae
Clinical picture Guillain Barre?
- Numbness followed by ascending weakness
- Absent DTRs or hyporeflexia
- Can get dysautonomia
