Neurology Sid Flashcards
What do you know about age of onset and sex of kids with absence seizures?
Eped: children >5 years, girls>boys,
What is the modified sarnat score for neonatal HIE?
What are the clinical features of tension headaches?
-dull/aching, bilateral: frontal or band-like
Acute treatment of tension headaches?
- Reassurance + rest + + Treat stress/anxiety/depression
- Wean caffeine
- Tylenol/ibuprofen
…Avoid opioids when possible
Migraine Epedimiology?
- Most frequent type of headache in children:
- 5% of school aged children
- 50% spont remission after age 10
Aura = cortical spreading depression (CSD)
What are the 5 criteria for Migraine without aura?
No of attacks, duration, headache features, asociated Sx and no other Dx likely
How many attacks do you need to have to Dx Migraine with aura?
A. . >2 attacks
How do you abort migraine?
- Rest in dark, quiet room
- ibuprofen probably more effective. Acetaminophen monotherapy not recommended
- Gravol or IV metaclopromide for nausea
- Triptans (eg sumatriptan) = 5-HT agonists to abort if above ineffective in adolescents
N.B. Caffeine not recommended in children
Non-pharm prophylaxis: Migraine prevention?
Non-pharm prophylaxis:
1. Food diary, avoid TRIGGERS, Nutraceuticals (magnesium, riboflavin, butterbur)
2. Behavior mgmt: biofeedback, self-hypnosis, relaxation technique
How do you treat Status migrainosus?
Status migrainosus (>3 d):
- Dark, quiet room, IVF, sedation
- IV NSAID (Keterolac)
- Chlorpromazine or Prochlorperazine IV is highly effective
What is the Epid of cluster headaches?
(Trigeminal Autonomic Cephalalgias)
Epid: Mainly boys, onset after 10y, rare
How doyou treat cluster headaches?
Acute:
- Oxygen inhalation
- Triptans
- Steroids
Chronic
Lithium
What are the Pseudotumour cerebri presure ICP limits for infants and children?
Characterized by increased ICP
- > 200 mm H2O in infants;
- > 250 mm H2O in children
Broad classes of Etiology of pseudotumour cerebri ?
MIDHV…idiot
Metabolic:
Infectious:
Drugs:
Hematologic:
Vascular:“
idiopathic intracranial hypertension”
General management of PTC?
- identification and treatment of underlying cause
- If due to obesity -weight loss
- If due to drug stop drug! - For most patients, repeated follow-up and monitoring of visual acuity is all that is required
- The initial lumbar tap may that follows a CT/MRI is diagnostic and can be therapeutic
What do you know about Epedimiology of Infantile spasms (West syndrome)?
Epid: Peak onset - 3 to 8 months, 90% < 1 year.
What do you know about Cyanotic breath-holding spells age of onset and disappearance?
Peak - 2 yr of age, abate by 5-8 yr , rare < 6 mths
What do you know about triggers for Pallid breath-holding spells?
painful experience, such as falling and striking the head or a sudden startle.
What do you know about Masturbation/self-stimulating behavior Eped- age, sex and situations?
GIRLS, 2 months and 3 yo, when bored
How do you define Medication overuse headaches?
Medication overuse - OTC medications more than 3 times per week for 3 mths
Indications for Neuroimaging in headaches?
Indications for Neuroimaging - questionable v1
Indications for Neuroimaging - questionable v2
What are the causes of floppy baby with central hypotonia?
What are the causes for Central Hypotonia?
What are the features of Peripheral Hypotonia?
What are the causes of Peripheral Hypotonia?
Juvenile myoclonic epilepsy (“of Janz”) Epedimiology?
Eped: adolescence, AD , chromosome 6 with variable penetrance
What no of attacks do you need to Dx migraine without aura?
> 5
What duration is needed to Dx migraine without aura?
1-72 hrs
What headache features are needed to Dx migraine without aura?
C. > 2 of:
- Unilateral
- Pulsating quality
- Mod or severe intensity
- Aggravation by routine px activity
What other features are needed to Dx migraine without aura?
D. During HA, > 1 of
- Nausea, vx
- Photo/phonophobia
E. Not attributable to another disorder
What % of Px with migraine without aura have a family Hx?
90%
What are the clical features of abscence seizures?
no aura
-
- no post-ictal state.
- Can provokein the office by hyperventilation.
What is the treatment for abscence seizures?
- 1st line Ethosuximide.
- If GTC..Valproic
Recent peds in review series on seizure types and management
· Since 40-50% of children with absence seizures will have GTC seizures, VPA optimal choice as it covers for GTC as well.
· Carbamazepine first-line for partial seizures
What are the associations with tension headaches?
- -NO nausea/vomiting
- assoc with anxiety/depression
- onset later in day (AM rare)..-wax/wane
How do you Dx tension heachaes?
- Dx by exlusion Hx and exam
- In teeenagers, difficult to DDx from migraine
Which form of migraine is more common..no aura or with aura (classic)?
No aura
When does the aura present before a migraine?
Migraine HA begins during/within 1h
Fx of Typical aura?
- Visual aberrations/dysesthesias/speech (5-60 min)
- retinal migraine variant: blindness/scotoma
Fx of Hemiplegic Aura?
Unilateral sensory/motor signs, ±aphasia
Fx of Basilar-type aura?
Vertigo, ataxia, tinnitus, vision change
What are the 3 broad Dx features of migraine with aura?
> 2 attacks, aura, Not attributed to another disorder
What are the triggers to migraine?
stress, fatigue, anxiety, exercise, menstrual, food
Indications for Drug treatment to prevent migraine?
For > 2-4 HA per month or unable to attend school regularly
•
What are the drug Rx for prevention of migraine?
- Flunarazine (CCB) 5 mg daily (only proven therapy)
- Propranolol (BB)
- Others: Anticonvulsants (Topamax), amitriptyline, cyproheptadine, Sandomigran (Pizotifen)
What is the Modified Raskin Protocol (adults) For status migainosus?
-DHE (dihydroergotamine), Metoclopromide, ±steroids
What is the pattern of attacks with cluster headaches?
- Cluster of daily attacks for 2-12 wk,
- few times per year
- seasonal
What are the symptoms of cluster headaches?
- Unilateral, sharp/burning,
- worse supine,
Associated Sx of cluster headaches?
- assoc with coryza,
- autonomic phenomena (tearing & sweating),
Eye Sx of cluster headaches?
periorbital edema,
ipsilateral ptosis
-conjunctivitis,
Pseudotumor cerebri CSF details?
Normal CSF cell count, CSF protein,
Pseudotumor cerebri MRI details?
normal ventricular size/anatomy/position
Pathophysiology of Pseudotumor cerebri?
abnormal CSF production, ¯absorption, edema, abnormal vasomotor control, venous obstruction
PTC What are the Metabolic/Endocrine and Drug causes of pseudotumour cerebri?
- Metabolic: galactosemia
- Endocrine:, hypoPTH, Addison, pregnancy
- Drugs: Prolonged steroids, Vitamin A, Retinoic acid
Infectious pseudotumour cerebri ?
- Viral: roseola, sinusitis, Guillan-Barré
- ENT: Otitis Media, mastoiditis,
Drugs pseudotumour cerebri ?
- Antibiotics; doxycycline, tetracycline
- Acne: isotretinoin
- ? OCP
Hematologic pseudotumour cerebri ?
: polycythemia, hemolytic anemia, iron-deficiency
Vascular - pseudotumour cerebri ?
: Venous sinus thrombosis
Clicial Fx of PTC?
- Headache (most frequent)-Pressure type (e.g., am, worse after laying down, better with standing, papilledema)
- Vomiting also occurs (but not as bad as for posterior fossa tumour)
- Diplopia (secondary to paralysis of abducens (CN VI) nerve)
- Most patients are alert and lack constitutional symptoms
Pseudotumor cerebri exam signs?
- bulging fontanelle;
2.percussion of skull = ‘cracked pot sound’
- papilledema is most consistent sign in a child beyond infancy
- …N.B. a focal neurological sign suggests a process other than pseudotumour cerebri
Complications:
Pseudotumor cerebri cplxns?
Optic atrophy and blindness
Preventative Rx of tension headaches
• Non-pharmacological (regular meals & sleep, avoid
caffeine, avoid food triggers, exercise)
• Limit use of abortive therapies to <15 x per month
Medications for PTC?
- Acetazolamide, furosemide
- Corticosteroid
- Considering treating sinus thrombosis with anticoagulation
**Keep in mind a slow-growing tumour or obstruction of a venous sinus
Clinical Fx of infantile spasms?
Clin:
- brief Frequent clusters of flexor, extensor, or mixed spasms
(neck, trunk, arm muscles)
- then a phase of sustained muscle contraction lasting 2 - 10 seconds.
- Mostly when awakening from or going to sleep.
- Many clusters occur each day.
Inv of infantile spasms?
EEG
- Waking state -hypsarrhythmia, is dramatically abnormal, consisting of high-voltage slow waves, spikes, and polyspikes accompanied by background disorganization.
- During sleep-Burst suppression patterns.
Infantile spasms mimics?
1.Infantile colic
with flexion of thighs and crying Infantile spasms have a poor prognosis. The etiology is not determined in 40% of children.
Etiology/Progx of infantile spasms?
- Idiopathic (40% N development < fits) has a better response to therapy, 40% have a good IQ. Etiology is determined in 60% (Table 181-4).
- symptomatic group: Poor response to Rx and lower IQ (Tuberous sclerosisor Downs)
Rx of infantile spasms?
- vigabatrin- visual field deficits (retinal toxicity)
- ACTH, oral prednisone (S/E-Irritability, , hypertension, glycosuria, infections, adrenal axis effects
What is the nromal trigger for Cyanotic breatholding spells?
TRIGGER: Usually predictable and is always provoked by upsetting or scolding an infant.
Clinical Sx of Cyanotic breatholding spells
brief, shrill cry, forced expiration and apnea.
- cyanosis and LOC , -/- GTC fits, opisthotonos, and bradycardia, may be repeated within few hrs-
Cyanotic breatholding spells Inv?
TESTS: (EEG) is normal.
-
Cyanotic breatholding spells management?
- support and reassurance, be consistentand not reinforce the child’s behavior after the child recovers
- placing the child in bed NOT cuddle, play, or hold the child for a given period of time until recovery is complete
- consider treatment with iron supplementation
Cyanotic breatholding spells
Pallid breatholding spell Sx and Inv?
EVENT:
apnea, LOC, becomes pale and hypotonic, +/- GTC
-Bradycardia with periods of asystole of longer than 2 sec may be recorded.
TESTS EEG is normal.
Pallid Breatholding spells management
- conservative measures as for cyanotic spells
- trial of atropine sulfate 0.01 mg/kg/24hr in divided doses with a maximum daily dose of 0.4 mg,which increases the heart rate by blocking the vagus nerve, may be considered in refractory cases.
N.B. Atropine should not be prescribed during very hot weather because an episode of hyperpyrexia may be initiated
What are the 5 self stimulation features?
- Repetitive stereotyped episodes:..Sudden onset, usually persists for minutes
- Tonic posturing with copulatory movements, adduction of thighs
- Without manual stimulation of genitalia
- Autonomic: Flushed skin, sweating, grunting
- NO LOC
Self stimulation management - exam?
looks for evidence of sexual abuse or abnormalities of the perineum
Self stimulation management?
Reassurance - will subside by 3 years of age, no specific therapy
What do you know about Benign focal epilepsy - Epedimiology?
Age onset, Incidence, % of afebrile efits, Fam Hx
- Age 5 and 10 years
- incidence may be 21 per 100,000
- 16% of all afebrile seizures
- FamHx -13%
N.B…..
Clinc:
- focal seizure- face and arm
- during sleep or on awakening in more than half of patients.
- abnormal movementor sensationaround the face and mouth with droolingand a rhythmic guttural sound. Speechand swallowingare impaired.
- Usually only have seizures at night
• May have less than 10 events
EEG: -epileptiform activity in centro-temporal regions (either unilateral or bilateral)
Mangt/Progx:
- May need anticonvulsant therapy- Keppra (levetiracetam) appears to be best
- IQ and brain MRI normal
- resolves after puberty.
What do you know about Benign focal epilepsy - clinical Sx
- focal seizure- face and arm
- abnormal movement or sensation around the face and mouth with drooling and a rhythmic guttural sound.
- Speech and swallowing are impaired.
EEG: -epileptiform activity in centro-temporal regions (either unilateral or bilateral)
Mangt/Progx:
- May need anticonvulsant therapy- Keppra (levetiracetam) appears to be best
- IQ and brain MRI normal
- resolves after puberty.
What do you know about Benign focal epilepsy - when occur and number of events
- May have less than 10 events
- Usually only have seizures at night, during sleep or on awakening in more than half of patients.
What do you know about Benign focal epilepsy- Inv?
EEG: -epileptiform activity in centro-temporal regions (either unilateral or bilateral) with centro temporal spikes
Bening focal epilepsy management + prognosis?
- May need anticonvulsant therapy- Keppra (levetiracetam) appears to be best. Can use Carbamezepine.
- IQ and brain MRI normal
- Resolves after puberty.
N.B. Treatment was associated with a reduction in generalized but not focal seizures
Prognosis in absence epilepsy?
- 60% fit free 9 yrs after seizure onset
- Non-remitters may go onto Juvenile Myoclonic epilepsy
-
What are the factors increasing chance of no remission in absence epilepsy?
- Cognitive difficulties at onset
- absence Status epilepticus before or during Rx
- GTC or Myoclonic fits after Rx onset
- Abnormal background on initial EEG
- Hx generalized fits in 1st degree relatives
Juvenile myoclonic epilepsy- clincial Fx, what types of seizures?
- Morning myoclonic seizures
- Absence
- Eventually GTC fits
Juvenile myoclonic epilepsy- Mangt?
1. Keppra OR valproic acid, but therapy must be maintained for life.
When does Juvenile myoclonic epilepsy occur?
morning myoclonus occurring predominantly within 90 minutes of awakening.
What is Episodic migraine?
a disorder of recurrent attacks,
What is the definition of chronic migraine?
- headaches on ≥15 days per month for ≥3 months, with
- ≥8 days per month meeting criteria for migraine.
What are the clinical features of Peripheral Hypotonia?
1. CNS:
Bright and alert, NO seizures or deficits like eye movement problems
2. Muscles:
Profound weakness and hypotonia + paucity of movements in antigravity muscles
3. Reflexes:
Depressed or absent reflexes
What are the 4 phases of migraine headaches with aura?
- the premonitory phase
- aura/headache phase
- postdrome phase
What are the premonitory symptoms of migraine?
- CNS: fatigue, irritability, social withdrawal, food cravings
- Face: pallor or shadows under the eyes
- GUT: urinary or bowel changes,
What is the change in the brain that causes an aura?
a cortical spreading depression…key clinical features of aura are spread and progression.
What are the + and -ve Sx of an aura?
- positive symptoms (scintillations or tingling) followed by
- negative symptoms (scotoma or numbness)
How fast does the migraine aura spread and how do the Sx change?
- spread over at least 5 minutes
- progression from one symptom to the next (eg, from visual to sensory)
What are the autonomic Sx in migraine?
ENT: sense of aural fullness, Nasal congestion and/or rhinorrhea
Skin: Facial sweating and flushing
Eyes: Lacrimation, Conjunctival injection, Ptosis, miosis
Periorbital edema
What are the features of migraine postdrome?
CNS: elation, fatigue, cognitive difficulties, pallor, ,
GIT: thirst, anorexia, food craving
Eyes/Skin: visual disturbances, ocular pain,paraesthesia,
What are criteria for diagnosing migraine without aura?
What is the criteria for the Dx of migraine with aura?
Specific features of the aura Sx: ( at least 3/6 features
-
Timing of aura Sx
a. At least x1 aura Sx spread gradually over > 5 minutes
b. > 2 or more aura Sx occur in succession
c. Each aura Sx last 5-60 mins - At least 1 aura Sx is Unilateral or Positive
3. The Aura is accompanied/followed within 60 minutes by a headache by a headache
When should a head MRI be done in Px with nonacute headache?
●Rapidly increasing headache frequency
●History of lack of coordination
●History of localized neurologic signs or subjective numbness or tingling
●History of headache causing awakening from sleep (although this can occur with migraine headache)
What are risk factors in chronic headaches?
- medication overuse
- Major depression
What are the features of Medication Overuse headache? •
OTC medications more than 15 times per month
- Triptans more than 10 times per month
- Opioids more than 10 times per month
- Causing headaches on at least 15 days per month
- For At least 3 months
Febrile fits What are the risk factors for developing Febrile fits?
1. Hx of febrile fit:
a. 30% if child has 2 or more febrile fits
b. first or second degree relative with a history of
febrile seizures (10-20% if sib, greater if parent)
- developmental delay
- attendance at day care
- hx of >28 days in NICU
Febrile fits What factors increase risk for recurrence of Febrile fits?
- young age (50% recurrence when < 12 months)
- • family history of febrile seizures
- • initiated by low fever
- • persistent neurologic abnormalities
- • first febrile seizure was complex
Febrile fits How does the nature of the fit change the risk of epilepsy in the future?
Risks for Developing Epilepsy
- general population = 0.5%
- simple febrile seizure = 1% (2x baseline)
• complex febrile…
– multiple in 24 hrs = 4% (8x baseline)
– prolonged (>15 min) = 6% (12x baseline)
– focality = 30% (60x baseline)
– 2 features present = 25% (50x baseline)
– 3 features present = 50% (100x baseline)
Unprovoked Seizures What is the Risks of Recurrence?
• risk of recurrence after
- 1 unprovoked seizure = 40%;
- after 2 unprovoked = 80%
- after focal seizure in child with delay and epileptiform discharges on EEG = 80-90%
- 75% of recurrences happen within first 6 months
