Neurology Flashcards
Clinical signs of hydrocephalus
- Bulging fontanel
- Sundowning
- Poor feeding/vomiting
- Lethargy
- Increasing head circumference if fontanels are not closed
- Stridor/apnea, vocal cord dysfunction
What are classic signs of Juvenile myoclonic epilepsy (Janz Syndrome)?
• EEG: 4-6 Hz spike and slow wave, polyspike and slow wave discharges
- Presentation is in early adolescence; myoclonic jerks in the morning, causing patient to drop things
- Generalized tonic clonic seizures upon awakening
- Juvenile absence seizures
- Triggers: Sleep deprivation, alcohol, photic stimulation
What is the most common cause of acute onset ataixa?
Acute cerebellar ataxia
suspected autoimmune mechanism in response to viral infection 2-3 weeks after initial illness
A child presents at age 2 with ataxia that does not self resolve, difficulty with horizontal gaze and frequent sino-pulmonary infections. What is the suspected condition?
Ataxia telangiectasia (though the telangiectasias may not present until middle childhood)
Must confirm with alpha-fetoprotein and genetic testing
A child presents with lower limb weakness, ataxia, nystagmus, absent DTR and hypertrophic cardiomyopathy. What is the suspected condition and what is the genetic basis behind this condition?
Friedrich’s Ataxia
Trinucleotide repeat expansion (GAA) which results in oxidative stress injury in the mitochondria of cells of the spinocerebellar tracts, dorsal columns in the spinal cord, the pyramidal tracts, and the cerebellum and medulla
In which situations is antiepileptic medication less likely to be discontinued?
- Mesial temporal lobe sclerosis
- Lennox-gastaut
- Severe myoclonic epilepsy
What adverse effects are to be expected for Carbemazepine?
- Hyponatremia
- SJS/allergy
- Aplastic anemia/leukopenia
- Neural tube defects if mother is pregnant
What adverse effects are expected for Valproic acid?
- Hepatotoxicity
- Hair loss
- Pancreatitis
- Thrombocytopenia
- Weight gain
What adverse effects are expected for Topiramate?
- Kidney stones
- Anhydrosis
- Parasthesias
- Weight loss
- Mental slowing
- Glaucoma
What adverse effects are expected for Clobazam?
- Hyperactivity
- Drowsiness
- Drooling
What adverse effects are expected for Lamotrigene?
- Rash
* SJS/TENs
What adverse effects are expected for Levetiracetam?
• Aggressive behaviour
What adverse effects are expected for Phenytoin?
- Gingival hypertrophy
- Hypertrichosis
- Fetal Hydantoin Syndrome (Fetal Dilantin Syndrome)
- Interactions with Septra (decreased clearance)
What is the classic triad expected for botulism?
- Intact sensorum
- Descending paralysis
- Absence of parasthesias
Will also see: parasympathetic involvement i.e. dry mucous membranes, constipation, blood pressure instability, urinary retention
What treatment is required for suspected botulinum toxicity?
- IV Ig botulinum toxin within 3 days
- Supportive management to maintain respiration and safe nutrition
- Fecal botulism spores/toxin detection for confirmation
What treatments are available for treatment of sialorrhea?
- Supportive: Bibs, bandanas, etc
- SLP: Oral motor skills & sensory awareness (#1 most effective if >cognitive abilities
- Anticholinergics 1st line
- Opthalmic atropine drops SL
- Glycopyrrolate (enteral)
- Transdermal scopolamine patches
AE: Urinary retention, vomiting, constipation, blurry vision, xerostomia (dry mouth); intolerance in 30%
- Surgical: If mod-severe, unresponsive to Tx for 6+mo:
- Botox injections
- Salivary duct ligation, ablation, duct removal
What nerves are involved in an Erb Palsy?
Erb-Duchenne-Type Injury (C5, C6 +/- C7 = “plus”)
“Waiter’s tip” position - limp arm, pronated wrist, internal rotation
• Most common type
• Consider phrenic nerve injury (“C3, 4, 5 keeps the diaphragm alive”)
What nerves are involved in a Klumpke Palsy?
Klumpke-Dejerine Injury (C8-T1)
- Sensory (ulnar side of hand) deficit
- “holding the towel” position - paralysis of extensors of forearm & flexors of wrist & intrinsic muscles of hand :. Absent grasp
- +/- Horner syndrome (if 1st thoracic root involved) & cervical sympathetic damage – ipsilateral ptosis & miosis
What is the prognosis for most brachial plexis injuries?
- Erb’s Palsy - most likely spontaneous resolution
- Klumpke Palsy - most likely spontaneous resolution; seek surgery if not improved by 3 months
- Phrenic nerve palsy - most likely spontaneous resolution; seek surgery if not improved by 3 months
What are the 2 types of breath holding spells?
1) Pallid
• caused by reflex vagal-cardiac bradycardia and asystole
• Child usually has “silent” cry with marked pallor
2) Cyanotic / “blue”
• Does not occur during inspiration; results from prolonged expiratory apnea and intrapulmonary shunting
What is the most common form of craniosynostosis?
Premature closure of the sagittal suture, resulting in long skull i.e. dolicocephaly/scaphocephaly
• More common in boys
What syndromes are associated with craniosynostosis?
- Apert syndrome - coronal suture closure
* Crouzon syndrome - clover-leaf, mostly F, closure of coronal sutures
What are the features of a febrile seizure?
- Pt is between 6 months and 6 years of age
- Generalized tonic-clonic seizure
- lasts 15 mins
- only one seizure within 24 hours
What is the change that a child with a febrile seizure will develop epilepsy?
- 2-7% of children with febrile seizure will develop epilepsy later in life (Nelson’s)
- 1-2% risk as per Uptodate
What are the risk factors for recurrence of febrile seizures?
- Major: age <1 yr, duration of fever <24 hrs, fever 38-39 C
* Minor: fmhx of febrile sz, fmhx of epilepsy, complex febrile sz, daycare, M, low serum Na
Which infectious agents are more likely to cause Guillain Barre Syndrome?
- Campylobacter jejuni
- H. flu for Miller-Fisher syndrome
- Mycoplasma pneumoniae
What features on LP are to be expected in a case of Guillain Barre?
- High protein (up to twice the upper limit of normal
- No WBC/pleocytosis
- Normal glucose
- Negative bacterial/viral cultures
What is Miller Fischer Syndrome?
Rare variant where instead of ascending weakness, patients have external/internalopthalmoplegia, ataxia and areflexia with distal paresthesias