Neurology Flashcards
First line treatment of infantile spasms
Vigabatrin (in Canada)
ACTH (in USA)
Prednisolone (used at ACH)
Best treatment for infantile spasms in patient with tuberous sclerosis
Vigabatrin
Most important side effect of vigabatrin
Retinal toxicity
Investigations to consider for frequent sleepwalking and/or safety concerns
PSG
Ferritin
If safety/QOL concerns with sleepwalking in absence of underlying trigger, what treatment to consider
Benzo
Two types of breath holding spells and what investigation to consider
Cyanotic and pallor
Serum ferritin
CSF abnormality in anti-NMDA receptor encephalitis
Lymphocytic pleocytosis
First line treatment for pituitary adenoma
Dopamine agonist
VPA side effects (x3)
Hepatoxicity
Thrombocytopenia
Pancreatitis
You must look for _____ in all presentations of opsoclonus-myoclonus syndrome. What investigation would you start with first?
Neuroblastoma
Abdo ultrasound
What gene mutation are you looking for in Rett Syndrome?
MECP2 gene mutation
Newborn with arm paralysis and Horner Syndrome
Total brachial plexus palsy
Duchenne Muscular Dystrophy - x2 important physical exam findings
+Gower’s sign
Calf pseudo-hypertrophy
Duchenne Muscular Dystrophy
a) age of onset
b) age of loss of ambulation
a) 2 years old
b) 12 years old
Difference between DMD and BMD from a pathophysiology perspective?
DMD - complete absence of dystrophin
BMD - inactivation and decreased production of dystrophin
Systemic manifestations of DMD?
CNS: Intellectual impairment CVS: Cardiomyopathy RESP: Impaired pulmonary function GI: Decreased gastric motility MSK: Fractures, progressive scoliosis
Classic GI infection leading to Guillain Barre Syndrome as well as the first line treatment
Campylobacter jejuni
IVIG
What medication can you give to patients with behavioural side effects secondary to Keppra?
Pyridoxine (vitamin B6)
Major risk factors for recurrence of febrile seizures
Age <1 years, fever duration <24 hours, fever 38-39deg
Erb’s Palsy - distribution
C5-C6 injury
Klumpke’s paralysis - distribution
C7-T1 injury
EEG finding for Benign Rolandic Epilepsy
Centrotemporal spikes
Causes of thunderclap headache
- Vascular: SAH, CVST, stroke, RCVS
- Structural: third ventricular colloid cyst, 3/4th ventricle tumour
- Infectious: meningitis, encephalitis
- Primary headache
What type of epilepsy would you think about in a child with febrile status epilepticus?
Dravet Syndrome
Criteria for a Complex Febrile Seizure
- Age <6 months or >5 years
- 15+ minute duration
- Focal
- Multiple in 24h
What criteria should you use for Sydenhams?
Jones Criteria
Major Jones criteria
Carditis Arthritis Chorea Erythema marginatum Subcutaneous nodules
What is the diagnosis if a child with GAS infection followed by OCD and tics?
Sydenhams
Genetic transmission of Huntington’s
Autosomal dominant
Difference between OCD and tics and stereotypies?
OCD - or else something bad is going to happen
Tic - feel an urge
Stereotypies - occur when they are excited
Criteria for Tourette Syndrome
- Multiple motor
- At least 1 vocal tic
- Occurs for >1 year
What should you screen for when adolescent comes in with tics?
Substances (Substance induced tic disorder)
What are the first line medications for tics (motor/vocal)? What class of meds would you consider next?
Clonidine, guanfacine
Then anti-psychotics
Ataxia telangiectasia - what two things can you see in addition to ataxia and derm
Tumors + sinopulmonary infections
Most common cause of stroke in neonates
Embolic - placenta, cardiac (PFO, other CHD), umbilical catheter
Three most common causes of childhood ischemic stroke
- Arteriopathy (Sickle cell, arterial dissection, moya moya)
- Cardiac disease (CHD)
- Thrombophilia
Two most common causes of childhood hemorrhagic stroke
- Structural (aneurysm, AVM, tumor)
- Hematologic (hemophilia)
Primary + secondary prevention for SCD related strokes
Primary - hydroxyurea
Secondary - transfusions
Secondary causes of Moya Moya
Sickle cell, TB meningitis, cranial radiation, NF1, Turner Syndrome, T21, CHD, PHACE, Alagille syndrome
Migraine criteria
A. At least 5 attacks fulfilling B-D within last year
B. Headache attacks lasting 1-72 hours - when untreated
C. At least 2 characteristics: a. Unilateral location (for children can be bilateral), b. Pulsating quality, c. Moderate to severe intensity, d. Aggravation by or causing avoidance of routine physical activity
D. During headache at least one: a. Nausea and/or vomiting, b. Photo AND phonophobia
E. Not better explained by another diagnosis
x2 most common causes of ataxia in a healthy child
Drug ingestion
Acute post-infectious cerebellitis
Normal LP opening pressure (by age range) and when to be concerned for intracranial HTN
- Normal: Neonate (8-10), 1m-4y (1-10), 8y-adol (10-20)
- Concerning: >25
Ddx for toe walking (x7)
- CP
- Congenital shortening of Achilles
- Spinal dysraphism
- Equinovarus deformity
- Intraspinal + filum terminale tumor
- Hereditary/acquired polyneuropathy
- Muscular dystrophy
Definition of status epilepticus
= continuous generalized tonic-clonic seizure activity with LOC for longer than 30 minutes
= OR >2 discrete seizures without return to baseline mental status
Pre-hospital medication given for seizure lasting >5 minutes
IN midaz 0.2 mg/kg (max 5mg/nare)
In-hospital first-line medication for seizure + when to move on to second-line
- First-line: IV midaz/loraz 0.1 mg/kg (max 4-5mg)
- Next: Can given 1st line again 5 min later. Once at least 2 doses of benzo’s have been given (including pre-hospital) then move on to 2nd line
Second line options for status epilepticus (including dosing)
- Fospheny 20mg/kg
- Phenytoin 20mg/kg
- Keppra 60mg/kg
- Phenobarb 20mg/kg
Next steps to consider when giving second line options for status epilepticus
- Can given a second dose of 2nd line - do NOT mix pheny with fospheny
- If 2 or more doses of second line, then consult specialty services and consider alternative airway
- Consider dose of pyroxidine 100mg IV for infants <18 months
Presentation of increased ICP in infants vs children
- Infants = macrocephaly, splayed sutures, apnea, bradycardia, desats, sundowning, bulging fontanel, irritable, shrill cry, FTT
- Children = AM/night-time headache, emesis, aLOC, anorexia, papilledema, CN 3 + 6 palsy (diplopia), personality difference
Idiopathic intracranial HTN - symptoms, exam, and investigations
- Symptoms: HA, fatigue, N/V, anorexia, stiff neck, diplopia
- Exam: papilledema, 3rd + 6th nerve palsy, visual field constriction
- Ix: normal CT head, elevated opening pressure, normal CSF profile
Idiopathic intracranial HTN - etiologies
- 90% idiopathic
- Drugs: tetracycline, nitrofurantoin, CC’s, excess vitamin A
- Endo: obesity, hyperthyroidism, Cushing syndrome, hypoparathyroidism
- Thrombosis: of dural venous sinuses from head trauma, AOM, mastoiditis, obstruction of jugular veins in SVC syndrome
What AED can make absence seizures worse?
-Carbazepine
What AEDs (x3) to consider for absence seizures?
Ethosuximide, VPA, lamotrigine
Prognosis for absence epilepsy
75% will have a complete remission by adolescence
What is the most common epilepsy syndrome?
Benign epilepsy with centro-temporal spikes (Rolandic)
x2 AED’s not to use for primary generalized epilepsy?
- Carbazepine (will worsen)
- Phenytoin (could worsen)
Why do we not use phenytoin or fosphenytoin as a routine anticonvulsant?
Gum hypertrophy
Red flags for a secondary headache
- Thunderclap
- Occipital/valsalva = Chiari I
- Wakening in AM, papilledema, diplopia = inc ICP, intracranial hypertension
- With standing = spontaneous intracranial hypotension
- Recent head injury
- Known coagulopathy/thrombophilia
- New headache + risk of thrombosis (SLE, nephrotic syndrome)
- Pituitary symptoms = short stature+obesity, new bed wetting, vision loss
Characteristics of a medication overuse headache
- Daily or near daily (>15 days per month)
- Present upon wakening
- Relieved by medication
- Recurs later in the day
How much medicine is required to lead to medication overuse headaches?
- NSAIDs or tylenol >15x/month for at least 3 months
- Triptans >10x/month for at least 3 months
Most common type of Spinal Muscular Atrophy?
Type 1
Best gross motor developmental milestones achieved by each type of SMA
- Type 1: Never sit
- Type 2: Sit but never walk
- Type 3: Can walk
Typical timeline for symptom onset amongst SMA types
- Type 1: <6 months
- Type 2: 6-18 months
- Type 3: >18 months
How many SMN2 copy numbers for each SMA type?
- Type 1: 2
- Type 2: 3
- Type 3: 4
What to think of for hammer toes
Charcot Marie Tooth
Presentation for Charcot Marie Tooth
- Delayed GM milestones
- Tight heel cords (clumsy, toe walker)
- Hypotonia
- Weakness
- Self-mutilation
Exam findings for Charcot Marie Tooth
- Abnormal gait + clumsiness
- Foot deformities (pes cavus, hammer toes)
- Weakness
- Hypotonia
- Foot drop
- Evidence of self-mutilation
First line testing for Charcot Marie Tooth
Genetic testing
Difference between muscular dystrophy and congenital myopathy?
- Dystrophy = problem with muscle fibre integrity = high CK
- Myopathy = abnormal function of muscle = normal CK
Who is cognitively normal (a) congenital myotonic dystrophy or (b) congenital myopathy?
B
Muscle disorder with anticipation
Congenital myotonic dystrophy
3 types of congenital myopathies and the main features
- Nemaline rod: bulbar dysfunction
- Central core: malignant hyperthermia
- Centronuclear: eye + face weakness
adenomatous sebaceum - what dx and other name?
TSC
Angiofibromas
What intracranial finding is spastic diplegia associated with?
Bilateral PVL
What GA timeframe does PVL occur in?
24-32 weeks
x2 unique physical exam features for myotonic dystrophy?
- grip myotonia = clenched fist, then try to relax but takes several seconds to do so
- percussion myotonia = to thenar prominence will cause flexion of hand
What to be concerned about if patient having seizures, hypertension, oral facial movements, emotional lability/confusion?
Anti-NMDA encephalitis
-Orofacial dyskinesias
What does dopamine agonist do for pituitary adenoma?
Decrease prolactin
Decrease size of adenoma
Criteria for low risk BRUE
- > 2 months old
- Not less than 32 weeks GA
- Not more than one event
- No CPR
- Event <1 min
- No concerns on history or exam
What investigation is important to do for neuroblastoma?
Urine VMA + HVA
Dx for myoclonus (+/- generalized involvement) typically occurring in the morning?
Juvenile Myclonic Epilepsy
What kind of neurological presentation is botulism + what scary presentation typically happens first?
- Symmetric, descending, flaccid paralysis
- Begins with CN involvement with bulbar palsies = poor feeding, poor suck, drooling, and weak cry
What should you give for a patient with Guillain Barre and what should you definitely not give?
- Give IVIG
- Do NOT give steroids
What kind of neurological presentation is Guillain Barre?
-Areflexic, ascending, flaccid paralysis
What is the most specific indicator for neonatal seizures?
Eye movements
What would be on the differential for a neuromuscular blockade type presentation similar to myasthenia gravis in a 6-12 month old?
Botulinism
What happens to pupils in botulinism?
Mydriasis - poor reactivity
Risk factors for recurrence for a child with first presentation of a febrile seizure - Major + Minor
Major:
- Age <1yr
- Fever duration <24hr
- Fever 38-39 degrees
Minor:
- Family history of febrile sz or epilepsy
- Complex febrile sz
- Day care
- Male
- Low serum sodium
What reflex is intact for a child with Erb’s palsy?
Grasp reflex
When do we get worried about a sacral dimple and consider U/S?
- Multiple dimples
- Diameter >5mm
- > 2.5cm above anus
- Dimple outside sacrococcygeal region
- Cannot see the bottom
- Leaking fluid, hair, or colour change
In terms of etiology, what do you think about hemiplegia CP vs spastic diplegia CP?
(a) perinatal insult
(b) prematurity, PVL
When does regression of development occur for patients with adrenoleukodystrophy?
~4 years of age
Shape for intracranial bleeds - (a) epidural and (b) subdural
(a) lens
(b) crescent
x2 treatments for GBS - +1 tx NOT to use
IVIG
Exchange transfusion
NOT steroids
Bells palsy tx - with doses
pred 2mg/kg x5 days then taper
Basilar migraine sx
- Diplopia
- Vertigo
- Tinnitus
- Bilateral paresthesias
- Dysarthria
