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Juvenile Myoclonic Epilepsy


10% of epilepsy / presents usually at 15-16 yrs

Treatment: VPA, Lamictal, others under study

Course: this type of seizure disorder rarely remits spontaneously
Febrile seizures

Peak 6 months to 3 years (maximum 3 months to 5 years) / cause developmental delay

Treatment: phenobarbital, ACTH / ?treat underlying cause of fever + Tylenol
Infantile spasms (Salaam attacks)

< 2 yrs / poor long term neurological outcome (developmental delay) / massive flexor spasms / no loss of consciousness / occur in clusters upon awakening

EEG: hypsarrhythmias



Treatment: respond to ACTH (unclear if helpful in long term outcome?)
Lennox-Gastaut

frequent, mixed, generalized – refractory to treatment (try a ketogenic diet)


Alcoholic Seizures (and Alcoholic Withdrawal)
Tremulousness

Withdrawal seizures
Alcoholic hallucinosis
Delirium Tremens (DTs)

2-4 days after cessation / lasts a few days / mortality 5%


Treatment:

  • supportive measures

  • long-acting benzodiazepines for 42-78 hrs

  • B12, thiamine (give thiamine before glucose to avoid acute Wernicke encephalopathy



Headaches
Red flags: change in pattern, progression, first severe or worst ever, abrupt onset, awakening from sleep, symptoms > 1 hr, onset at age < 5 yrs or > 50 yrs, in setting of other serious medical disease, altered consciousness (ICH), triggered by exertion/valsalva
Primary (90%)

tension > migraine >> cluster



Depression


HA often the first sign of depression / treat with TCA (SSRI’s thought less effective with this type of depression/HA)
HTN

usually diastolic > 115 in order to cause HA


Other

meningitis, trauma (CVA, ICH), venous sinus thrombosis, drug side effect, glaucoma


Migraine Headaches

Incidence in US (15-20%), familial (70-80%) / major undiagnosed problem


Presentation: pulsatile, throbbing, unilateral (usually) / duration 1-2 days

  • nausea (90%), vomiting (60%), diarrhea (15%), photophobia (80%), phonophobia, light-headed (70%), vertigo (30%), paresthesia (30%), scalp tenderness (70%), visual (40%, 10% fortification spectrum), seizure (4%), confused (4%), syncope (10%), may have cranial autonomic features (tearing or nasal congestion)

  • migraine aura without headache (20% migraneurs and 40% of aura positives)

  • fortification spectrum – central scotoma, migrating scotoma with peripheral scintillation, colored

  • Note: migraine often wakes people from sleep (don’t think such HA automatically implies brain tumor)

  • Inciting factors: certain smells, foods (10% can identify), stress, falling barometric pressure

Mechanism (proposed): dysfunction of central neurogenic regulation / vascular reactivity / serotonin? / dorsal raphe nucleus / wave of 20-30% hypoperfusion progressing occipito-frontally, usually not crossing midline at 2-3 mm/min, lasting 4-6 hrs / prevention: type 2 antagonists / abortion: type 1 agonists

Complications: possible cause of lipid abnormalities and early CVA
Menstrual migraines

occur during menses and are often refractory to therapy / Amerge (naratriptan) seems to be most effective (1 mg bid 2-3 days prior to menses and a week after) / pregnancy and menopause may produce remission


Vertebrobasilar migraines (hemiplegic, ophthalmic)

more rare / post-HA confusion up to 5 days


Transformed migraines – low grade HA that just doesn’t go away (many days) / Treat same as other migraines / toradol IM
Mixed headache syndrome – chronic tension headaches and periodic migraines
Treatment: (see prevention below)  any one agent should have > 60% efficacy


    • Triptans – newer triptans (Maxalt, Zomig) are very effective, but very expensive ($20/pill)

    • caffeine, ergot, butalbital (this is addictive)

    • Other: biofeedback / acetominophen/dichloralphenazone/isometheptene (2 then 1 or 2 caps an hour later) / ergotamine + caffeine or triptan / rapid acting NSAIDS taken in large doses and repeated a few hours later (meclofenamate, naproxen, ibuprofen) / steroids may provide relief / prochlorperazine IV (sedation may help) / butalbital + ASA or Tylenol (often overused) / metoclopramide (Reglan) given 20 mins before vasoconstrictive agents and NSAIDS to control nausea / consider prevention with several episodes per month


Prevention:

B-blockers 1st line / failure does not preclude trying a different B-blocker

Tricyclic antidepressants 1st line

Anti-epileptics 1st/2nd line

Topamax et al

Divalproex (VPA) low doses and titrate upward

Tegretol


?Gabapentin (Neurontin)

NSAIDS can be given for several weeks and are often effective for prevention / toradol (IM) can often abort refractory migraine
Calcium channel blockers 2nd line may take 6-8 weeks for effective control

Methysergide ? 3rd line may cause retroperitoneal fibrosis

Botulinum toxin type A injections into forehead muscle / studies ongoing
Tension Headaches
Pathology: muscle spasms and/or central neurogenic regulation

Ddx: analgesic and caffeine withdrawal headaches

Presentation: tightness and pressure in entire head / may have associated tightness/spasm in neck and shoulders / may also have nausea and photophobia (non-specific)
Treatment:

biofeedback/relaxation/physical therapy / tricyclics +/- NSAIDs (amitriptyline, doxepin for increased sedation, cyclobenzaprine for sedation/muscle relaxation / 10-20 mg q hs up to 100 mg) / SSRI’s are less effective directly but may work indirectly by treating causative depression / muscle relaxants (chlorzoxazone, orphenadrine) / carisoprodol (muscle relaxant/sedative which is metabolized to meprobamate, can cause dependency) / aspirin/caffeine/orphenadrine combination (may cause rebound headache) / analgesics such as codeine, propoxyphene, oxycodone, hydrocodone frequently overused but may be the only effective means of relief


Cluster Headaches

men 8x > women / usually younger men



Pathology: hypothalamus involved?

Ddx: sinusitis, trigeminal neuralgia

Presentation: less than 3 hrs duration (usu. 20 mins to 2 hours) / intensely painful, unilateral, in or behind eye (maybe ½ of cases, pain is somewhere else such as ipsilateral suboccipital area), often occur more at night, can be precipitated by alcohol or vasodilating drugs / patients will usually pace to try to get relief (unlike migraine) / parasympathetic overactivity  20% with Horner’s-like syndrome (ipsilateral tearing and conjunctival injection > miosis, ptosis, cheek edema)
Treatment:

100% O2 at 7-8 L/min given only 10 min/h (50% effective)

verapamil 40-60 mg/d for several days, then tapered / prednisone given at the same time at 40-60 mg/d for several days, then tapered / lithium 300-900 mg/d / ?methysergide 4-8 mg/d / prednisone and methysergide work within 2-3 days, while verapamil and lithium work slowly

Others: NSAIDS / clonidine / ergotamine, SC sumatriptan or DHE may shorten attacks / under investigation: surgery, IV histamine
Cough Headaches

4:1 male to female / 25% with structural anomalies like Arnold-Chiari


Headache from Viral Illness

EBV, influenza, adenovirus, cold


Post lumbar puncture

bifrontal, occurs 1-2 days after LP / lasts 3-4 days


Trigeminal Neuralgia (Tic Douleureux)

This condition really sucks / irritation of trigeminal (or other) nerve that causes misfiring and sharp pain sensation (in spells)

Treatment:

Acute pain: tegretol 1st, baclofen 2nd, other (acupuncture) 3rd

Prevention: Trileptal

If medical treatment fails, can do surgery (for young patients, usu. successful) and radiofrequency ablation (for older patients) / these measures usu. work for 1-5 yrs


ICP (Intracranial Pressure)
Causes: NPH, PC, ACM, Chiari I, tonsillar herniation, intracranial AVM, sinus venous thrombosis

Cushing’s triad – bradycardia, hypertension, breathing changes

Treatment: mannitol, steroids, carbonic anhydrase inhibitor decreases CSF production (treats ICP) (watch HCO3 levels)


    • vasogenic edema (white matter)

    • cytotoxic edema (gray matter)

swelling of cells from hypoxia / hypoosmolality / Reye’s syndrome

    • interstitial edema

transudation of CSF from ventricles from hydrocephalus
Normal Pressure Hydrocephalus (NPH)

Dementia, incontinence, ataxia / usually  gait 1st, incontinence 2nd, dementia 3rd



  • DAT pathology seen in ⅓

  • apraxia  delayed initial step, wide-based (PD has more shuffling gate)

  • NPH can also have bradykinesia and bradyphrenia (slow thought)

Ddx: heavy metal poisoning, chronic ETOH

Diagnosis: radioisotope diffusion studies in CSF and Miller-Fisher test (removal of 30 mL CSF improves gait)

Treatment: decision of when to shunt is a (difficult) clinical decision / Miller-Fisher test is best predictor of successful treatment with VP shunting (works in ) / VP shunting has 40% complication rate (meningitis, shunt malfunction, subdural hematoma) / carbidopa/levodopa helps some patients who do not receive VP shunt
Pseudotumor Cerebri

benign, idiopathic increase in ICP / young, obese females



Presentation: headache, visual disturbance

Findings: papilledema, may have palsy of CN VI

Causes: tetracycline, vitamin A overdose, idiopathic

Note: if sudden onset (r/o saggital sinus thrombosis)

MRI: normal or small ventricles

Treatment: usually self-limited, can use steroids, reduce pressure with spinal taps, osmotic diuretics, oral glycerol?, surgery (rarely)
Arnold-Chiari Malformation

communicating hydrocephalus


Chiari I

positive Babinski, cerebellar signs (eye signs), spasticity / usually presents at young age (may present at older age occasionally)


Tonsillar herniation

Duret’s hemorrhages in mid-brain, pons and respiratory arrest (medulla)


Intracranial AVM’s

convulsions, increased ICP / often present with high-output congestive heart failure



Miscellaneous Neurological Problems




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