Neurology Flashcards
When do infantile spasms present?
Infantile Spasms
• 90% present in first year (peak 4-6
months)
What do spasms look like?
Frequent clusters of flexor, extensor, or
mixed spasms
What is the EEG pattern for infantile spasms?
EEG pattern hypsarrhythmia
- Chaotic, high amplitude background
- Multifocal spikes
How do you treat infantile spasms?
Treated with vigabatrin (retinal toxicity) or ACTH (irritability, central adrenal axis suppression)
or high dose Prednisone
What conditions are associated with Infantile Spasms?
Tuberous Sclerosis (e.g., hypopigmented macules) or Down Syndrome
What is sandifer syndrome?
Sandifer Syndrome
• Abnormal movements (often axial stiffening) occurring due to GERD
• Usually occur with or after feeds in “spitty” baby
What does benign myoclonus of infancy look like?
Benign Myoclonus of Infancy
• AKA Shuddering Attacks
• Sudden brief symmetrical axial flexor spasms of trunk and head lasting 1-2 s OR “vibratory” tonic flexion of neck
• May be provoked by excitement/fear
• Normal exam
• Spontaneous remission by 5 years of life
Need to R/O Infantile spasms
What is the EEG finding for benign myoclonus of infancy?
Normal
How old are children when they have breath holding spells
• Present from 6-18 months of age
What do breath holding spells look like and what are the two types?
- ~1 min episodes of LOC
- Cyanotic: Apnea and cyanosis after agitation
- Pallid: Limpness, diaphoresis and pallor after injury
What is the treatment for breath holding spells?
- 100% will resolve by 8 years of age
* Reassure parents and consider treatment with iron supplementation
What condition is breath holding spells associated with?
• Association with iron deficiency anemia
How old are children with infantile masturbation?
Infantile Masturbation
• Often starts within first year of life
• More common in girls
• Most have spontaneous remission by 3 years of life
What do episodes of infantile masturbation look like?
More common in girls
Characterized by episodes of:
• Repetitive pelvic rocking
• Adduction of legs
reduced responsiveness - but distractible…may return to behaviour
can get some autonomic symptoms with this may happen
• Pelvic pressure (hands or on object (e.g., car seat))
How old are children when Childhood absence epilepsy starts?
Childhood Absence Epilepsy
• Present in first decade (peak 6-7 years)
What does Childhood absence epilepsy look like clinically?
• Typical absence seizures hundreds of times/day
• Otherwise patient is essentially norma
- May be doing poorly in school….frequent seizures
What do you treat childhood absence epilepsy with?
Treat with ethosuximide: First Line
valproic acid or lamotrigine
What is the prognosis of Childhood Absence Epilepsy?
70% of patients will outgrow seizures in
adolescence
What should you NOT treat childhood absence epilepsy with?
oxcarbazamine should NOT be used in these patients - can make it worse
What is the typical age of onset for benign rolandic epilepsy?
4-10 years
What do seizures in BRE look like?
• Nocturnal focal seizures of face lasting 1-2 minutes with no loss of consciousness
- Often 2-3 hours after falling asleep.
- often involves vocal cord movement as well - so cannot speak unable to speak or swallow, but can communicate and can follow commands will see a lot of drooling
- Usually only have seizures at night
• May have less than 10 events total
What is the prognosis for BRE?
Overall a BENIGN condition • Most outgrow in puberty • Likely do not require treatment • If treat, Keppra (levetiracetam) appears to be best
What medication can you NOT use for BRE?
for those who require treatment (frequent seizures)
oxcarbazapine can make it worse in this case
phenytoin avoided because of long-term consequences
Would treat with carbamazepine
What anti epileptics are used in children <2
Children < 2 years • Phenobarbital • Good for generalized or focal • Major adverse effect = sedation • Reversible effects on IQ
- Second line
- Levetiracetam • Topiramate
What anti epileptics should you not use in children <2
Children < 2 years
• Don’t use…
• Valproate – increased risk of toxicity (hyperammonemia)
• Carbamazepine or oral phenytoin – poor absorption
What anti epileptics do you tend to use in children >2
- Valproate is never wrong (can use for generalized or focal): Unless the patient has an underlying metabolic disease (e.g., mitochondrial, glycogen storage disease)
- Avoid in teenage girls
What are side effects of valproic acid
• Valproate is never wrong • Often not first choice simply because of many risks associated with use • Sedation • Hair thinning • Weight gain • Tremor • Thromobocytopenia • Bone health • Pancreatitis • Hepatitis
Child > 2 with focal seizures…best anti epileptic?
Children > 2 years
• Focal onset
• Carbamazepine/oxcarbazepine
• Levetiracetam
Child > 2 with generalized seizures…best anti epileptic?
- Generalized onset
- Levetiracetam
- Lamotrigine
- Ethosuximide (absence)
What are the No-Nos of Antiepileptics?
No No’s
• Do not use carbamazepine/oxcarbazepine or phenytoin with absence or myoclonic seizures
• NEVER give phenytoin IM (can cause burns, necrosis
but you can give fosphenytoin IM)
• Do not use valproic acid in child with potential metabolic disease
Indications for Imaging a Headache
Definite
• New associated neurological findings (e.g., seizures, persistent abnormal gait)
• New focal abnormalities on neurological
exam
• Papilledema
Questionable
• Sudden onset of severe headache (worst pain of life)
• Pain that wakes patient from sleep
• Pain consistently worst in mornings
• Recent head injury
• Change in headache type
• Occipital location (think Chiari malformation)
• Pain worse with cough or valsalva
• Poorly defined pain (does not fit any
classic headache type)
• Persistent morning time vomiting with headache
Diagnostic Criteria for Paediatric Migraine
SULTANS Criteria: 1. 5 or more attacks n a year 2. Each lasts 1-72 hrs •Headache has 2 of the following characteristics... Severity (moderate-severe pain) UniLateral (or BL) Throbbing Aggravated by activity
• Plus at least one of…
Nausea/vomiting
Sensitivities = photophobia/phonophobia
• Not better explained by another condition
What does a hemiplegic migraine look like?
• Hemiplegic migraine
• Ca++ channelopathy
• Transient hemiplegia followed by headache
Family history of migraine
What is an abdominal migraine?
• Abdominal migraine • Non-specific abdominal symptoms • Usually in young children • Eventually develop classic migraine Usually a family history of migraine
What does a basilar migraine look like?
• Basilar migraine
• Transient ataxia and/or cranial nerve
deficits
posterior foss affected
What does a migraine with Benign paroxysmal vertigo look like?
• Transient episodes of severe vertigo lasting min-hrs +/- headache
- Family history of migraines
sudden attacks of falling to the ground
What does a Retinal migraine look like?
• Transient episodes of blindness or scotoma
Visual Vision loss with subsequent headache
Abortive Management of paediatric migraine.
Abortive
• Ibuprofen 10 mg/kg q 6 hrs (may require double strength at first dose)
• Triptans (sumatriptan, rizatriptan, zolmitriptan) – more adverse effects
• Acetaminophen (high dose)?
What are the preventative treatments for paediatric migraine?
Proven
• Avoid caffeine
• At least one hour of exercise per day
• Placebo
• Nutraceuticals (magnesium, riboflavin, butterbur, coenzyme Q10)
• Flunarizine 5 mg daily
Others: valproic acid, amitriptyline, topiramate, propranolol
Possible
• Non-pharmacological (regular meals & sleep, avoid food triggers, stress management)
if asthma is in the question, do NOT use propranolol can mask complications in patients who have asthma
What is the clinical presentation of intracranial hypertension or pseudotumor cerebra?
• Pressure type headache (e.g., morning time, worse after laying down, better with standing, papilledema)
- vomiting
- Transient visual obscuration and diplopia
- Most patients are alert and lack constitutional symptoms.
Examination:
- bulging fontanel
- a “cracked pot sound” or MacEwen sign (percussion of the skull produces a resonant sound)
- Papilledema with an enlarged blind spot
- An inferior nasal visual field defect may be detected
LP…Opening pressure >30
Causes of pseudotumor cerebri
• Idiopathic form – young obese females
• Secondary forms – tetracyclines (minacycline), venous sinus thrombosis
Growth hormone
Vitamin A
Treatment for IIH
**The key objective in management is recognition and treatment of the underlying cause.
Pseudotumor cerebri can be a self-limited condition, but optic atrophy and blindness are the most significant complications of untreated pseudotumor cerebri.
- weight loss regimen
- drug is thought to be responsible, it should be discontinued.
- serial monitoring of visual function is required.
- Serial determination of visual acuity, color vision, and visual fields is critical in this disease.
- Serial optic nerve examination is essential as well.
- Serial visual-evoked potentials are useful if the visual acuity cannot be reliably documented.
- The initial lumbar tap that follows a CT or MRI scan is diagnostic and may be therapeutic. Several additional lumbar taps and the removal of sufficient CSF to reduce the opening pressure by 50% occasionally lead to reso- lution of the process.
- Acetazolamide, 10-30 mg/kg/24 hr
- Corticosteroids are not routinely administered
- Sinus thrombosis is typically addressed by anti- coagulation therapy.
What are tension type headaches like?
everything that migraine is not
• Bilateral, pressure, mild intensity, not worse with activity
Tension Headache
• Bilateral location
•Pressing/tightening quality
•Mild-moderate intensity
• Not aggravated by routine physical activity
• No nausea/vomiting
• No more than one of photophobia or phonophobia
What is a Trigeminal Autonomic Cephalalgias?
- Includes cluster headache (and others)
- Rare in children
- Unilateral, severe, and associated with prominent autonomic phenomena (e.g., tearing, swelling)
What does SMA Type 1 look like clinically?
Spinal Muscular Atrophy
• Disease of anterior horn cell (motor neuron)
• Proximal weakness, hypotonia, areflexia (because the anterior horn cell can not conduct this reflex)
- severe hypotonia
- generalized weakness
- thin muscle mass
- absent tendon stretch reflexes
- involvement of the tongue, face, and jaw muscles; and sparing of extraocular muscles and sphincters.
- Diaphragmatic involvement is late.
- contractures, ranging from simple clubfoot to arthrogryposis
- infants lie flaccid with little movement, unable to overcome gravity
More than 65% of children die by 2 yr of age, and many die early in infancy.
What does SMA 2 look like?
- affected infants are usually able to suck and swallow, and respiration is adequate in early infancy.
- Nasal speech and problems with deglutition
- Scoliosis
Many survive into the school years or beyond, although confined to an electric wheelchair and severely handicapped.
The outstretched fingers of children with SMA often show a characteristic tremor owing to fasciculations and weakness.
Myalgias are not a feature of SMA.
The heart is not involved in SMA.
Intelligence is normal
Lab findings for SMA
CK level may be normal but more commonly is mildly elevated in the hundreds.
Results of motor nerve conduction studies are normal (an important feature distinguishing SMA from peripheral neuropathy)
Inheritance of Duchenne Muscular Dystrophy
Duchenne Muscular Dystrophy
• X-linked recessive – found in boys
Clinical Presentation of DMD
• Progressive destruction of muscle over time – does not present in infancy
• Proximal weakness, Gower’s, calf pseudohypertrophy (not atrophy)
- Poor head control in infancy may be the first sign of weakness.
- In later childhood, a “transverse” or horizontal smile may be seen.
- Walking is often accomplished at the normal age of about 12 mo, but hip girdle weakness may be seen in subtle form as early as the 2nd year.
- A Trendelenburg gait, or hip waddle, appears at this time.
- Common presentations in toddlers include delayed walking, falling, toe walking and trouble running or walking upstairs, developmental delay, and, less often, malignant hyperthermia after anesthesia.
- After the calves, the next most common site of muscular hypertrophy is the tongue, followed by muscles of the forearm.
- Cardiomyopathy, including persistent tachycardia and myocardial failure
Myotonic Dystrophy Clinical picture
Myotonic Dystrophy
• Trinucleotide repeat disorder (>50 CTG)
• Classically associated with “myopathic facies” (bilaterally non-fatigable ptosis, tented mouth)
• Present with weakness – myotonia later
• Multiple systems involved (cataracts, cardiac arrhythmias, balding, etc.)
h/o polyhydramnios, hypotonia, weakness involving face and limbs, contractures
• Severe respiratory issues (25% die in neonatal period)
What does Guillain-Barre Syndrome Look like?
• Post-infectious, sub-acute polyneuropathy
• Length dependent symptoms the longest nerves are affected first - they will accumulate injury first
- (“glove and stocking” by the time you have your stocking on (half way up your leg), you start to have arm involvement)
Must have:
• Progressive motor weakness of more than one limb
• Areflexia or marked hyporeflexia
Supportive:
• Progression over days to a few weeks
• Relative symmetry
• Mild sensory loss
• Onset with extremity pain or discomfort
• Cranial nerve involvement
• Onset of recovery 2-4 weeks after halt of progression
• Autonomic dysfunction
• Initial absence of fever
• Elevated CSF protein after 1 week of symptoms
• Abnormal nerve conduction studies with slowed conduction or prolonged F waves
Classic Infections associated with Guillain barre
- Classically after infection with campylobacter jejuni
* H. Influenzae is second most common in axonal GBS
Treatment for Guillain Barre Syndrome
• Treat with IVIG
Spastic hemiplegia usually presents how?
Spastic hemiplegia often isn’t seen until after 6 months of age
– presents with early hand preference
What type of CP is PVL associated with?
Spastic diplegia is associated with bilateral periventricular leukomalacia (PVL)
• PVL only occurs between 24-32 weeks gestation
What is Syndeham Chorea associated with?
- After group A strep infections, patients can present with Sydenham chorea
- Almost constant chaotic movements of limbs
- Behavioural changes
Signs and Symptoms of UMN lesion
• Upper Motor Neuron
– spasticity ( tone)
– hyperreflexia
– no fasciculations, no atrophy
Signs and Symptoms of LMN Lesion
• Lower Motor Neuron – low tone – hyporeflexia – atrophy – fasciculations
Peripheral Nerve Lesion Findings
• peripheral nerve
– motor or sensory to specific distribution (focal neuropathy, mononeuritis) or diffusely, but legs > arms
– weakness is distal > proximal
– LMN findings
Neuromuscular junction lesion findings
– pure motor problems – random or generalized distribution - Fatiguable weakness - normal or dec tone - normal reflexes
Muscle problem findings
– motor only
– weakness proximal > distal
– preserved reflexes (until late)
– atrophy
