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
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
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 ⅔) / 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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