1. Neuropathic pain - aching, shooting, throbbing, or burning.
2. Temperature sensations.
3. Autonomic dysfunction (cardiac arrhythmias, orthostatic hypotension, impotence, incontinence, and constipation).

• loss of vibration & proprioception, weakness, fasciculations, and loss of deep tendon reflexes.

BP p. 159

• Fat is bright on a T1-weighted image; water is dark.
• H₂O (including CSF) is bright on T2; T2 is most useful in evaluating the spinal cord. Think T₂O.
• Gadolinium is the contrast agent used in MRI, and usually used to obtain T1-weighted images.
• Flair is a strong T2-weighted image, but one in which signal from CSF has been inverted and is of low intensity rather than high; useful in identifying pathologic processes of the CNS, eg chronic lesion burden of MS.
• Susceptibility-weighted sequence is one that is sensitive to disruptions in the magnetic field (eg, calcium, bone, iron, hemosiderin). Areas of ↑ susceptibility appear black.
• DWI demonstrates cellular toxicity. Dx stroke, + w/in 30 min. Areas of restricted diffusion appear bright on DWI.

BluePrint p. 12

• usually follows a respiratory or GI infection (60-70%)j
• Campylobacter, CMV, EBV, HIV, Lyme, mycoplasma
• GBS is characterized by ascending paralysis and absent deep tendon reflexes.
• Many antiganglioside Ab can be found in GB (anti-GM₁, GD_1a, GQ_1b [+ in 90% of cases], GD_1b).
• GBS is an ascending motor paralysis with or w/o sensory disturbances.
• Albuminocytologic dissociation (↑ protein, ↓ cells); ↑ protein → xanthochromia; protein can get as high as 1 gram; CSF pressure normal.
• Ddx - transverse myelitis, NMJ problems, or myopathy.
• dx: first analyze CSF, then confirm with EMG.
• Tx - IV-Ig or plasmapheresis; steroids not beneficial.
• Miller-Fisher variant is characterized by gait ataxia, areflexia, and external ophthalmoplegia, usually w/o limb weakness.

BP p. 161, 162, PT p. 37, 308, Kap Q0697m

• aka chronic GBS.
• different from GBS in that it responds to steroids.
• 90% improve with tx, but 50% will relapse; tx often required for years.
• no response to steroids → IV-Ig or plasmapherisis.
• common in diabetes

BP p. 163, 127

• symmetric sensorimotor neuropathies that usually affects the legs more than arms; prominent large-fiber loss and sensory ataxia.
• 10% of peripheral neuropathies are associated with serum monoclonal gammopathy (M protein) that reacts with myelin-associated glycoprotein (MAG); primarily demyelinating.
• 1/3 of those pts have multiple myeloma, amyloidosis, macroglobulinemia, cryoglobulinemia, lymphoma, or leukemia.

BP p. 164

• neuropathic pain and dysesthesias (abnormal sensation).
• distal, symmetric, slowly progressive sensory loss; toes & feet before hands).
• autonomic insufficiency can be an important feature.
• weakness is a late feature.
• It is an axonal neuropathy involving small & large fibers.
• Glycemic control is best tx; duloxetine, gabapentin, pregabalin, or lamotrigine for pain.

BP p. 164

• uremic neuropathy (sensorimotor axonal degeneration → foot drop and leg weakness).
• porphyric neuropathy (parasthesias, dysesthesias, rapidly evolving weakness/paralysis [mimicking GBS], and with areflexia and abdominal pain); think about primidone (barbiturate can trigger acute intermittent porphyria).
• critical illness neuropathy (occurs in 50% of pts in ICU > 2 weeks).

BP p. 165, UW 3513

• Charcot-Marie-Tooth disease; most common hereditary neuropathy.
• CMT-2 is the only axonal motor neuropathy of CMT family; others are primarily demyelinating.
• Hereditary sensory & autonomic neuropathy (HSAN) is a hereditary neuropathy that affects autonomic sensory or motor nerves.

BP p. 165

• axonal damage, myelin changes, and nerve fiber loss.
• nerve hypertrophy often occurs; can be palpated.
• deep tendon reflexes are preserved.

BP p. 166

• rapidly progressive flaccid paraparesis (partial paralysis of lower limbs) w/ sphincter dysfunction, perineal sensory loss, lower limb areflexia.
• uncommon, but usually associated with CMV.

BP p. 166

• orthostatic hypotension
• diarrhea/constipation
• early satiety (gastroparesis)
• tachycardia/palpitations
• blurred vision
• urinary retention
• erectile dysfunction

BP p. 166

• Facial/CN VII - anterior 2/3
• Glossopharyngeal/CN IX - posterior 1/3

• essential tremor → cerebellum
• intention tremor (↑ tremor when near target) → cerebellum (overcorrection of motor error); spinocerebellar tract can be affected too.
• ET tx: propranolol (BP & HR can limit usage; other β-blockers not as effective); primidone (metabolized to phenobarbital); gabapentin; topiramate; botox.
• Resting Tremor (suspect parkinsons): sometimes tx with anticholinergic (trihexyphenidyl).

PT p. 24, Uptodate

• nystagmus ( rhythmic to-and-fro movements of eyes; has a fast component in one direction and a slow component in the other); evoked on gaze.
• ataxia
• dysarthria
• impaired judgement
• lethargy
• gingival hyperplasia
• bradycardia/arrhythmias
• agranulocytosis, leukopenia, pancytopenia
• hepatitis

PT p. 25

• facial nerve (CN VII) to the stapedius muscle.

PT p. 27

• loss of achilles tendon reflex
• subjuctive findings: pain in lower back or buttock region, often radiating down the posterior thigh and calf to the lateral and plantar surfaces of the foot and affecting the 4th and 5th digits of the foot.

PT p. 28

COWS - cold opposite, warm same

PT p. 29

superior cervical ganglion (post-ganglionic fiber) → travels along the common carotid → follows the internal carotid artery → follows the ophtalmic artery branch to the eye.

the sudomotor component to the forehead branches with the external carotid.

If a disease involves only the internal carotid artery → ptosis & miosis, but NO anhydrosis.

Occurence of painful horner syndrome is virtually diagnostic of carotid artery dissectoin.

PT p. 30

blood irritates the meninges

PT p. 31

bacterial meningitis; age and hx of probable URI, think pneumococcal meningitis.

PT p. 36

1. optic chiasm
2. pituitary gland
3. cerebral aqueduct (aqueduct of sylvius)
4. 4th ventricle
5. tentorium cerebelli
6. mamillary bodies

http://www.liv.ac.uk/HumanAnatomy/phd/mbchb/stroke/mri3.html

• communicating: mismatch between CSF production and absorption.
• non-communicating: blockage of the ventricular system.
• LP is safe in communicating hydrocephalus b/c pressure in ventricular & subarachnoid spaces are equilibrated with lumbar cistern.

PT p. 32

What is the target INR for a patient with atrial fibrillation?

antiphospholipid syndrome?

• INR 2-3; CHADS₂
• VTE: INR 2-3
• APS: INR 2-3 (UpToDate, Management of APS)
• Prosthetic Heart Valves: INR 2.5-3.5

UW3606

• lacunar stroke of the posterior limb of the internal capsule.
• lacunar stroke of the posteroventral nucleus of the lateral thalamus; during recovery paradoxical pain can result from diminished sensation/pain sensitivity (thalamic pain symdrome).
• lacunar strokes occur in hypertensive patients.

PT p. 51

• infarction involving the lateral medulla, which includes:
• nucleus and descending tract of the 5th nerve
• nucleus ambiguus (CN X)
• lateral spinothalamic tracts (pain & temp)
• inferior cerebellar peduncle
• descending sympathetic fibers
• vagus nerve
• glossopharyngeal nerve
• Symptoms: ipsilateral ataxia and ipsilateral Horner's; loss of pain & temp of face; injury to lateral spinal thalamic tract → contralateral pain & temp disturbances; dysphagia and dysphonia (weakness of palate, pharynx, vocal cords).
• usually due to occlusion of PICA, the last large branch of the vertebral artery.

PT p. 51, UW 4269

• afferents from CN VII, IX, and X; responsible for visceral sensation.
• projections are primarily parasympathetic and sympathetic pregangionic neurons in medulla and spinal cord.

PT p. 52

• amyloid angiopathy
• deposition of β-amyloid in blood vessels → disruption of vessel walls → predisposes to hemorrhage.

PT p. 52

postictal paralysis, a result of weakness after seizure activity; can last for hours to days. Cause is unknown, but could be 2° to neural exhaustion. However, it is necessary to r/o possible bleeding or new cortical damage w/ CT.

PT p. 54

• Sturge-Weber syndrome
• facial cutaneous angiomas and leptomeningeal angiomas.
• pt may be mentally retarded.
• often exhibits hemiparesis or hemiatrophy on the side opposite the port-wine nevus.
• seizures may develop.

PT p. 54

• HTN; very small and may be microscopic.
• common in perforating arteries (lenticulostriate).
• putamen is the most common site for hematoma formation.

PT p. 54

1. cortex around sylvian fissure (lateral fissure) and some frontal lobe structures involved in speech.
2. optic radiation running through temporal lobe → right homonymous hemianopsia.
3. left motor/sensory cortex.

PT p. 55

• CN III compression; aneurysm, tumor, etc.
• aneurysm in posterior communicating artery is especially likely to compress CN III; also superior cerebellar artery, posterior cerebral artery.

PT p. 55, 56

damage to the frontal eye field, which normally directs the eyes contralaterally.

PT p. 58

• transcortical motor aphasia
• similar to Broca's aphasia, but repetition is preserved.
• anatomically the lesion affects left frontal white matter and spares the overlying cortex.
• transcortical sensory aphasia is similar to Wernicke's apahasia, but repetition is preserved. 

PT p. 59

• classic is preceded by an aura of neurological dysfuntion.
• aura: often visual in nature, consisting of flashing lights, scintillating scotomas, or field cuts.
• both have hemicranial throbbing HA.

PT p. 81

• visual disturbances, can evolve to complete blindness.
• irritability may develop into psychosis.
• transient quadriplegia.
• stupor, syncope, and even coma may appear and persist for hours.

PT p. 81

• carbamazepine, phenytoin, gabapentin
• if pt does not respond to pharmthx → radiofrequency ablation.
• aka trigeminal neuralgia (injury to CN V)
• pain routinely triggered by cold stimuli.
• often associated with MS (aka symptomatic trigeminal neuralgia, b/c symptom of another condition), but also basilar artery aneurysms, acoustic schwannomas, and posterior fossa meningiomas.

PT p. 81, Kap imq42m

• a form of ataxia
• undershooting or overshooting of intended target with hand, arm, leg, or eyes.

• constant deep pain.
• often bilateral, but limited in its distribution (cheek, nose, zygomatic region) and can be unilateral.
• idiopathic, but responds to anti-depressants

PT p. 82

• amitriptyline HCL, verapamil, propranolol, valproate.
• prophylactic antiemetics: chlorpromazine, prochlorperazine, or metoclopramide.
• tx prophylactically when migraines occur at least 1-2x per month.
• triptans (5-HT₁ receptor agonists), isometheptene, ergotamine.

PT p. 82, 83, UW 3619

• ↓ dexterity (clumsiness)
• blurred vision
• bed wetting
• changes in speech
• gait instability

PT p. 84

• pain that originates in eye (retroorbital) and spreading over temple as HA develops; usually develops at night.
• HA accompanied by extreme irritability.
• lasts approx. 2 hours.
• autonomic phenomena: unilateral nasal congestion, tearing from 1 eye, conjunctival injection, ptosis & miosis (Horner's).
• prophylactic tx: verapamil, lithium, ergotamine, prednisone, indomethacin, cyproheptadine.
• tx: 100% O₂ and SQ sumatriptan.

PT p. 84, UW 4253, 3935

• idiopathic intracranial HTN, predominantly affecting obese women in their childbearing years; ↑ risk w/ OCP, corticosteroids, or tetracycline.
• pulsatile HA, transient visual obscurations, progressive visual loss, pulsatile tinnitus (whooshing sound), diplopia, shoulder & arm pain.
• papilledema or optic atrophy if longstanding.
• occasional 6th nerve palsy.
• other causes of ↑ intracranial pressure: SLE, renal disease, hypoparathyroidism, radical neck dissection, vitamin A intoxication, steroid withdrawal.
• dx: MRI to rule out any structural processes.
• tx: lumbar puncture, diuretics, acetazolamide, ventriculoperitoneal shunting, optic nerve sheath fenestration.
• rule out cerebral venous sinus thrombosis with MRV!!!

PT p. 85, Kap m000912m, UW 3162

• biceps brachii, brachialis, coracobrachialis
• carries sensory information from lateral cutaneous nerve of forearm.

PT p. 94

• Radial nerve; travels along spiral groove of humerus.
• commonly injured in alcoholics that have LOC in awkward positions → wrist drop.

PT p. 94

Due to the anatomy of the cranial cavity sudden deceleration damages the inferior frontal lobes and temporal lobes (coup injury).

occipital lobe (contrecoup).

PT p. 96

• the sensation of tingling radiating away from the percutaneous percussion of a peripheral nerve.
• in carpal tunnel → Tinel sign of median nerve at wrist.
• with carpal tunnel syndrome extreme dorsiflexion of wrist → pain.

PT p. 97, Kap Q0537m

• congenital motor neuron disease
• weakness, hypotonia, muscle atrophy
• lethal (weeks to months)
• destruction of anterior horn (LMN) → flaccid paralysis.
• it is one of the spinal muscular atrophies (SMA).

PT p. 220, BP p. 158

• encephalitis lethargica
• pt often develops transient fevers, lethargy, and HA.
• movement disorder develops w/ disease: chorea, athetosis, dystonia, myoclonus, oculogyric crises.
• sequela of disease is severe: unremitting parkinsonism.
• 1 in 4 patients die.

PT p. 114

• Facial (CN VII); 50%
• also II, III, IV, VI, VIII
• CN in cavernou sinus: III, IV, V (V₁ & V₂), VI.

PT p. 114

• anterior horn cells in spinal cord.

PT p. 115

The fluke itself does not invade spinal cord, but eggs are deposited into valveless veins of Batson (connects deep pelvic and thoracic veins to internal vertebral venous plexus). The patient develops granulomas around the ova that lodge in the spinal cord → compress spinal cord.

PT p. 115

• empiric tx w/; do not wait for LP results to tx!!!
• (< 3 mos) cefotaxime + ampicillin + vancomycin
• (3 mos to 50 yrs) ceftriaxone + vancomycin
• (> 50 yrs) ceftriaxone + ampicillin + vancomycin

Step-Up p. 371

• primary CNS lymphoma (EBV; confirm w/ CSF PCR); tumor may cause blindness via direct invasion of optic nerve.
• Toxoplasma gondii (most common cause of brain abscess in AIDS pt).
• other etiologies such as bacterial and fungal are possible.
• dx w/ LP
• tx Toxo w/ sulfadiazine and pyrimethamine

PT p. 116, 136

• Facial (CN VII)
• Bell's palsy
• tx w/ high dose penicillin or ceftriaxone IV for 10-14 days if meningeal irritation is present; if allx to IV tx w/ tetracycline for 30 days..

PT p. 119

• children and rarely seen after the age of 18.
• most affected children have had a bout of measles before the age of 2.
• disease may not present until 6-8 years after the initial infection; death occurs within 1-3 years.
• symptoms: deteriorating school performance, mood lability, intellectual deficits will develop, seizure, chorioretinitis, ataxia, hyperactive reflexes, + babinski.
• EEG: periodic bursts of high-voltage slow waves followed by recurrent low-voltage stretches (burst suppression pattern).
• CSF pattern similar to MS: ↑ γ-globulin fraction with presence of oligoclonal bands.
• measles appear to be directly responsible for demyelinating disease; significant portion of Ab is specific for measles.
• Eosiniphilic inclusions are typically present in cytoplasm.

PT p. 124

• ampicillin plus gentamicin

PT p. 122

• cat-scratch disease
• meningitis in immunocompetent
• encephalitis w/ status epilepticus and bacillary angiomatosis in HIV pts; BA can be found cutaneously and viscerally; caution w/ bx b/c they are prone to bleed.
• presents initially with regional adenitis (inflammation of lymph node or gland)
• MRI shows ↑ signal intensity in pulvinar (posterior thalamus).

PT p. 124, UW4373

• astrocytoma
• Grade 4 astrocytomas are called Glioblastoma Multiforme.
• also meningioma, oligodendroglioma

PT p. 132, Kap Q0144

• pilocytic astrocytoma
• medulloblastomas (arise in cerebellar vermis and grow down into 4th ventricle).
• ependymomas (grows as a mass filling 4th ventricle). 
• cerebellar gliomas
• found infratentorially (posterior fossa)

PT p. 132, Kap s2s353m, Q0146m

• thickening of the bone; most tumors cause bone erosion.
• calcification that can be seen on a skull x-ray suggests: astrocytoma, meningioma, oligodendroglioma.

PT p. 133

• angiomatoses (telangiectasias of retina).
• cysts of the liver, kidney, and pancreas.
• renal carcinoma
• hemangioblastoma in the brain (usually limited to the cerebellum or brainstem and occurs before renal carcinoma).
• erythrocytosis with cerebellar signs
• microscopic hematuria
• hepatosplenomegaly
• cerebellar tumors

PT p. 133, 217

breast, lung, kidney, skin, and uterus.

PT p. 134

• hypothalamic hamartoma
• acromegaly is also possible
• paroxysms of laughter are known as gelastic seizures.

PT p. 135

• loss or vertical gaze
• pupillary constriction with loss of light reflex
• lid retraction
• convergence-retraction nystagmus
• 2° to pineocytoma or tumors that occur in this region; syndrome occurs because of involvement of the dorsal midbrain in the region of the superior colliculus.

PT p. 137

• peripheral neuropathy
• symmetric, distal, mixed sensorimotor neuropathy
• neuropathy improves with dialysis

PT p. 149

• centrocecal scotoma
• the blindspot extends temporally to involve central vision.
• this can also be seen with thiamine deficiency (tobacco-alcohol amblyopia).

PT p. 151

• pyridoxine (vitamin B₆)
• peripheral neuropathy (pins and needles sensations, hypoactive reflexes, slightly impaired proprioception, but good strength).

PT p. 152

not 100% understood, but in smokers and alcoholics combined deficiencies in vitamin B₁, B₁₂, and riboflavin seem to cause tobacco-alcohol ambylopia (aka nutritional optic neuropathy).

PT p. 152

• pellagra, a nicotinic acid deficiency or tryptopahn deficiency,  which is its precursors
• dermatitis, diarrhea, dementia, and death
• occurs in people with diets limited to corn.
• Hartnup disease (intestinal malabsorption of tryptophan).

PT p. 153, 216

• progressive cramping of legs
• pins-and-needles sensation in feet & hands
• ataxia
• urinary incontinence
• spastic paresis
• severe vibration & proprioception loss
• absent deep tendon reflexes despite spasticity
• peripheral blood smea: hypersegmented polymorphonuclear leukocytes.

PT p. 144

• α tocopherol
• rare, but if it occurs it effects young children.
• ataxia
• limb weakness (proximal muscle weakness)
• absent deep tendon reflexes
• ophthalmoparesis
• poor pain perception in feet.
• ↑ CPK
• ↑ serum bilirubin
• sensory roots are especially vulnerable.

PT p. 153

inhibits neuronal migration

PT p. 221

• aka obesity hypoventilation syndrome
• obesity associated with hypersomnia AND sleep apnea.
• patient usually has hypoxemia and pulmonary HTN.
• smoking ↑ risk of developing syndrome.

PT p. 154

• post-traumatic seizure
• postictal confusion → improvement
• Todd paralysis (hemiparesis); however, hemorrhage/hematoma must be investigated
• seizure threshold ↓ when pt is asleep or sleep-deprived.

PT p. 155

CCl₄ is a potent hepatic toxin → ↑ ammonia levels.

PT p. 155

• general background slowing
• normal background rhythm in an adult is α activity at 8-12 Hz.

PT p. 164

• stimulus sensitive myoclonic jerks (limb or facial movements in response to loud noises).
• similar movements may develop with Huntington disease, but the movements are more fluid (chorea).
• early symptoms: ataxia, clumsiness, dysarthria, diplopia, distorted vision, blurred vision, field defects, changes in color perception, and visual agnosia.

PT p. 165, 167

• Alzheimer Disease
• as much as 50% of the dementia in the general population.

PT p. 165

• Progressive Multifocal Leukoencephalopathy
• caused by JC virus

• memory impairment
• aphasia (transcortical sensory aphasia)
• apraxia
• neuropsychiatric impairment
• mood disturbances
• delusions
• hallucinations
• personality Δ
• behavior disturbances

PT p. 166

• episode of complete and reversible anterograde and retrograde memory loss lasting up to 24 hours.
• patients have persistent loss of memory for time of attack.
• patients retain personal identity and can perform complex cognitive and motor tasks.
• occurs in middle-aged or older men and often occurs in setting of an emotional or other stressor (physical or sexual exertion).

PT p. 166

• involuntary movement disorder that occurs during pregnancy and involves relatively rapid and fluid, but not rhythmic, limb and trunk movements.
• may appear with estrogen use, but the fundamental problem is a dramatic change in the hormonal environment of the brain.
• problem dissapears with delivery or termination of estrogen use.

PT p. 179

• 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP)
• also von Economo encephalitis (encephalitis lethargica) from influenza epidemic of 1918-1926.

PT p. 179, 180

• ↑ writhing and jerking movements of the limbs.
• dopaminergic drugs can unmask chorea.
• other drugs: bromocriptine and lisuride.

PT p. 180

• intracytoplasmic
• eosinophilic inclusions with poorly staining halos around them.

PT p. 181

• dopamine cannot cross the blood-brain barrier.
• carbidopa also cannot cross the BBB.
• levodopa/carbidopa can cause hallucinations.

PT p. 182, 183, UW 3715

• benztropine, diphenhydramine, trihexyphenidyl.
• EPS (dystonia, pseudoparkinsonism, akathisia).

PT p. 182

• a form of focal dystonia
• blepharospasm (eyelid spasm)
• forceful jaw opening
• lip retraction
• torticollis
• tongue thrusting
• tx with Botox
• onset during 50s, but can be side effect of phenothiazine or butyrophenone (class eg, haloperidol).

PT p. 183

• Wilson disease
• ataxia, dysarthria, dyskinesia, parkinsonism, cognitive dysfunction.
• mood & personality Δ's.
• hepatic fibrosis → renal tubular acidosis
• tx penicillamine (toxic), treintine w/ zinc (less toxic).
• dx: ↑ 24 hour urinary copper excretion is most sensitive screening test.

PT p. 184, BP p. 118

• excessive sleepiness
• slowing of movements
• mild depression
• proximal muscle weakness
• bones, groans (abdominal), stones, and psychiatric moans (lethargy, fatigue, depression, dementia, coma).

PT p. 178

• passive flexion of neck → electrical sensation radiating down spine, signifying spinal cord disease.
• can occur in MS
• in MS massive Babinski response may → bladder evacuation.

PT p. 195

• in an MS flare use corticosteroids; GI disturbance is a side effect of steroids, add ranitidine to ↓ SE.
• interferon and glatiramer are not helpful during a flare.
• MS affects 1/1000 people.

PT p. 196, 197

• BK, JC, and SV40
• think Progressive Multifocal Leukoencephalopathy in pts w/ lymphoma, leukemia, or AIDS.

PT p. 197

• N-acetylaspartic acid metabolism
• → developmental regression at about 6 months of age.
• Δ's in brain white matter → spongiform appearance
• ↑ in brain volume and weight (macrocephaly).

PT p. 197

• spastic (UMN) bladder → premature bladder emptying
• little or no residual urine in bladder after  emptying b/c bladder contractility is good (distensibility is poor).
• patient usually has urgency or incontinence.

PT p. 198

• derivative of GABA; GABA_B receptor agonist.
• tx spasticity

• ↑ temperature
• heat sensitivity is called Uhthoff phenomenon
• summer, hot shower, or even exercise induced symptoms → consider MS.

PT p. 198

• after infxn (chicken pox, measles) or vaccine (rabies, small pox).
• examination of the brain reveals damage to small blood vessels and perivascular tissues in the white matter of cerebral hemispheres.
• dx suggested by MRI or CT with rapidly evolving white matter damage w/ ↑ ESR, ↑ CSF pressure w/ ↑ RBC/WBC/protein.
• difficult to differentiate from MS if it is the first MS attack.

PT p. 199, Kap m000690

• adrenoleukodystrophy
• X-linked disorder of ATP-binding transporter in peroxisomal system responsible for long-chain fatty acid metabolism → accumulation of long-chain fatty acid in adrenal cortical cells.
• symptoms of adrenal insufficiency: fatigue, weakness, hypotension, orthostasis, muscle aches, hypoglycemia, confusion, N&V, diarrhea

PT p. 199

• port-wine stain (nevus flammeus)
• ipsilateral leptomeningeal angiomas
• pheochromocytomas
• glaucoma
• seizures
• hemiparesis
• MR
• head CT → calcifications in cerebral cortex in railroad track pattern.

FA p. 467, PT p. 206

• H - hamartomas of the skin
• A - adenoma sebaceum (cutaneous angiofibromas, red patches on face containing many blood vessels).
• M - mitral regurgitation
• A - ash leaf spots
• R - cardiac rhabdomyoma
• T - tuberous sclerosis
• O - autOsomal dominant
• M - MR
• A - renal angiomyolipoma
• S - seizures
• also retinal phakomas (gliomatous tumors that require no tx and are principal criterion for making dx of tuberous sclerosis).
• CNS calcifications; calcified subependymal glial nodules; can → obstructive hydrocephalus.
• infantile spasms (may be confused with colic); tx w/ ACTH IM gel (6-8 weeks of tx)

FA p. 467, PT p. 219, 220, Kap m000365m

• cafe-au-lait spots
• lisch nodules (pigmented iris hamartomas)
• neurofibromas in skin (short sessile or pedunculated lesions)
• optic gliomas
• pheochromocytomas, meningiomas
• chrom 17
• macrocephaly, short stature, feeding problems, and learning disabilities.
• bony abnormalities - sphenoid dysplasia, congenital pseudoarthritis, scoliosis.

FA p. 467, PT p. 275

• Metachromatic leukodystrophy
• due to arylsulfatase A deficiency
• leads to impaired production of myelin sheath.
• difficulty walking, ↓ reflexes, dysarthria, ↓ mental functioning.

FA p. 466

• TS has no hepatosplenomegaly
• both have cherry red spot on macula, neurodegenerative disorder.
• TS - hexosaminidase A deficiency → GM₂ accumulation.
• NP - sphingomyelinase def → sphingomyelin accumulation.

FA p. 116

• microsomal TG transfer protein defect → VLDL formation problem → ↓ vitamin E delivery to PNS & CNS.
• ataxia
• peripheral neuropathy
• retinitis pigmentosa
• acanthocytosis (spurred/spiked/crenated red cells) due to impaired lipid transport for cell membranes.

PT p. 217

• men have hyperextensible joints and prominent thumbs, large ears, high-arched palate, hypotelorism (abnormally closed eyes), large tests.
• half the women with fragile X may develop MR.

PT p. 214, 216

• type I (adult) - cerebellar tonsils extend below foramen magnum; not symptomatic until adults. symptoms are referable to the cerebellum.
• type II - cerebellar anatomy more deranged; small posterior fossa → downward displacement of vermis & medulla below foramen magnum → hydrocephalus and MR; symptomatic at birth of infancy. Also associated with myelomeningocele & syringomyelia.

PT p. 222, Kap Q0257m

• dermatomyositis
• occurs as a paraneoplastic syndrome in 15% of cases (lung, ovaries, GI, breast, but usually not CNS tumors).
• heliotrope rash, erythematous rash across knuckles and at base of nails. may be associated with flat-topped purplish nodules over elbows and knees.

PT p. 229, 230

• Triad of cerebellar defects (ataxia), spider angiomas (telangiectasias), IgA deficiency.
• poor smooth pursuit of objects with eyes.
• Defect in ATM gene (DNA repair) → ↑ cancer risk (lymphoma, leukemia, radiation sensitivity).

PT p. 238

• ↑ serum levels of CPK
• X-linked (occurs in 1/3000 males)
• males often die before reaching sexual maturity; however, the gene incidence remains stable.

PT p. 231

• problems relaxing grip
• hypersomnolence
• premature baldness
• testicular atrophy
• cataracts

PT p. 232

numerous abnormally small muscle fibers intermingled with hypertrophied muscle fibers.

PT p. 227

• anterior horn cells (LMN)
• motor nuclei of brainstem
• large motor neurons (Betz cells, UMN)
• corticospinal tracts
• brainstem disease (diaphragmatic weakness, facial fasciculations)
• fasciculations of the tongue denotes a poor prognosis (basically involvement of musculature innervated by the cranial nerves).

PT p. 233

• the superior vermis of the cerebellum → gait instability and limb ataxia.
• specifically the purkinje cells.

PT p. 239

• damages both upper & lower motor neurons.
• antichlinesterase activity → HA, vomitting, abdominal cramps, excessive sweating, wheezing, twitching.

PT p. 239

• toxic levels → brain edema → ↑ ICP
• chronic: ataxia, tremor, psychomotor retardation, MR.

PT p. 239

• tremors (limb & facial)
• memory disturbances
• personality Δ (irritability)
• ataxia
• ↓ pain & temperature sense in feet.
• think Mad Hatter from Alice in Wonderland (felt worker or pulp/paper plants).

PT p. 236

• radial nerves
• bilateral neuropathies (abdominal pain, constipation, anemia, basophilic stippling, linear discoloration along the gingival margins (lead lines).
• wrist drop is common.
• tx w/ penicillamine

PT p. 240

• damage to posterior columns & dorsal roots.
• ergot is a potent vasoconstrictor.

PT p. 241

• manganese inhalation
• clinical picture is similar to Wison's disease (hepatolenticular degeneration).
• neuronal loss is prominent in globus pallidus, putamen, caudate, hypothalamus, and cerebellum.

PT p. 241

• development of extrapyramidal symptoms (parkinsonian gait & bradykinesia)
• delayed neurological deterioration is associated with CO poisoning (occurs 1-3 weeks after initial event).

PT p. 241

• tick paralysis
• removal of tick → remarkable resolution
• rapidity of progression & absence of ↑ CSF protein makes GBS unlikely.
• holycyclotoxin interferes with presynaptic release at the NMJ.

PT p. 242

• Hereditary degenerative disease involving retinal receptors and adjacent pigment cells.
• as degeneration progresses small accumulations of pgiment appear about the periphery of the retina.
• RP is associated with abetalipoproteinemia, Refsum disease, and other metabolic disorders.

PT p. 258

• Sturge-Weber syndrome
• chromosome 3

PT p. 259

• visual loss is substantial with papillitis, which is an inflammation of the optic nerve head.
• pts with papillitis also have pain on moving the globe and sensitivity to light pressure on the globe
• papillitis is often a sign of MS.

PT p. 259

• conversion disorder or malingering
• tunnel vision has no physiologic pattern of visual loss.
• pts w/ tunnel vision will report same size field even as test screen is moved farther away.

PT p. 260

• vitamin A deficiency
• retinitis pigmentosa

PT p. 261

• microaneurysms
• proliferative retinopathy

PT p. 261

• infection (Gradenigo syndrome)
• it is an osteomyelitis of the petrous pyramid
• abducens & trigeminal nerves are affected as they pass the tip of the petrous bone.
• chronic ear infection may extend to the petrous pyramid.

PT p. 262

• trochlear muscle b/c the trochlear nerve (CN IV) shares a nerve sheath with the ophthalmic division of the trigeminal nerve.

PT p. 262

• The parasympathetic fibers on the exterior of the nerve are supplied by a separate set of vessels that are usually unaffected by DM.
• The vessel usually affected by DM lies deep in the 3rd nerve.

PT p. 262

• pontine damage
• it is an involuntary movement of the eyes (rapid downward deviation followed by slow upward movement)

• Adie tonic pupil
• benign phenomenon
• however, consider drug use

PT p. 264

• AION
• damage to optic nerve from insufficient blood supply.
• visual defect is usually inferior altitudinal (above or below the horizon) defect with involvement of central vision.

PT p. 265 

• ossicles or cochlear nerve
• each ear is represented bilaterally even at the level of the brainstem!
• each cochlear nucleus projects to the temporal lobes bilaterally.

PT p. 273

• if it extends supratentorially → temporal lobe → wernicke's aphasia (fluent aphasia).
• infratentorially → cerebellum → ataxia, vertigo, N&V, morning HA.
• the lesions are usually abscesses formed by bacteria.

PT p. 274

• cochlea (↑ energy shears receptor filaments from hair cells).
• this can lead to high-tone sencorineural loss.
• sound energy is not strong enough to perforate eardrum.

PT p. 274

• PICA (cerebellum)
• vertebral artery (lateral medulla, Wallenburg syndrome).

PT p. 274

• lower tones are more susceptible
• tinnitus, vertigo, progressive hearing loss (can be unilateral).
• tx: low salt diet, dimenhydrinate, cyclizine, meclizine.

PT p. 274, Kap Q0846m

• Aspirin
• etOH
• quinine
• aminoglycosides

PT p. 274

Aspirin

PT p. 275

• β-2-transferrin; a CSF specific marker
• meningitis is the most frequent and severe complication of CSF leak.
• #1 cause of meningitis: S. pneumoniae, #2 H. influenzae.

Kap m000221m

• unique among the senses b/c it does not send fibers through the thalamus.

PT p. 275

• olfactory groove meningioma
• aka Foster-Kennedy syndrome
• meningioma extends posteriorly to involve ipsilateral optive nerve → compression. compression of nerve sheath prevents transmission of ↑ ICP down optic nerve; therefore, papilledema is only visualized on the contralteral eye.

PT p. 276

• damage to the long thoracic nerve (C5, C6, C7) which innervates the serratus anterior muscle.

PT p. 289

• fasciculations, fibrillations, flaccid paralysis, and hyporeflexia.
• lesions at the anterior horn cells or distally.

PT p. 290

• medulla (decussate as the internal arcuate fibers and ascend in the medial lemniscus).
• medulla (decussate at the pyramids).

PT p. 290, 294

• atherosclerosis
• syphilis (aortic arch)

PT p. 290

• artery of Adamkiewicz
• aka arteria radicularis magna
• enters approximately at T10 to L1.
• at risk during AAA repair.
• upper segments of spinal cord are usually supplied off vertebral arteries.

PT p. 291

As the syrinx expands it compresses the anterior horn cells of the cervical spine → weakness in distribution of affected neurons. However, the corticospinal tract is relatively unaffected by syrinx b/c it lies at the periphery of the cord → lower limb strength intact.

PT p. 292

• transverse myelitis
• can occur with MS, sarcoid, or viral & parasitic infections.

PT p. 293 

• it is a non-compressive sciatica
• sciatic nerve injury → foot drop (common peroneal nerve → deep peroneal nerve → anterior tibial muscle); irritation of spinal roots to sciatic nerve can occur with Lyme radiculopathy.

PT p. 306

• diabetes → transient weakness (LMN).
• individual nerves are transiently disabled in this disorder.

PT p. 307

• injury to C5 and C6 spinal roots.
• eg, birth trauma (head & shoulder forced apart).

PT p. 308

• spinal cord
• both dorsal and ventral spinocerebellar tracts are involved.
• posterior columns and lateral corticospinal tracts are also involved.
• Chrom 9 (frataxin), recessive

PT p. 308

• diabetes in 10% of pts.
• cardiac conduction system
• optic atrophy (1° to Friedreich or 2° to DM)

PT p. 308

loss of cells in the dorsal root ganglia → hyporeflexia

PT p. 309

• brachial plexopathy
• acute onset of pain in neck, shoulder, or upper arm.
• then rapid evolution of weakness of proximal arm.
• sensory loss also occurs.
• some cases are bilateral.
• cause unknown, but seems to occur after vaccination or viral illness; familial cases also occur with dominant pattern.

PT p. 310

• small-fiber neuropathy (pain & temp, para/sympathetic)
• absence of papillae on tongue
• recessive, primarily Jewish children.

PT p. 310

• barbiturates, phenytoin, sulfonamoide abx, estrogens
• tx - IV hematin

PT p. 311

• nitrous oxide poisoning
• suspect in health care professional w/ numbness, paresthesias, limb spasticity, ataxia.

PT p. 311

• sarcoidosis
• remember MS is a strictly CNS disease!

PT p. 311

painful sensory neuropathy

PT p. 311

• indistinguishable on MRI
• ADEM is, however, monophasic (one acute episode).

PT p. 324

• loss of bladder & bowel control
• paraparesis or paraplegia
• back pain
• saddle anesthesia
• sciatica
• this is a spinal nerve root issue
• caused by: trauma, lumbar disk herniation, malignancy, abscess.
• emergent MRI to dx!

PT p. 325, UW 4392

100% O₂ thx

PT p. 325

• sympathetic innervation of the iris.
• if a pt presents w/ anisocoria, but the iris color is different → congenital anomaly.

PT p. 317

• diffuse axonal swelling affecting white matter, corpus callosum, and upper brainstem.
• these foci are usually hemorrhagic.

PT p. 327

hypothermia

PT p. 328

• 74%
• 15% in men
• optic neuritis presents with unilateral visual loss and pain with eye movement. also loss of color vision and afferent pupillary defect. most commonly fundoscopic exam is normal; however, may present with swollen optic disc (papillitis) with a flame-shaped hemorrhage.

PT p.  329, Kap m000489m, m000613m

• corticospinal tract disease
• UMN problem → spastic bladder
• oxybutynin or imipramine (both have anticholinergic effects).

PT p. 329

protracted seizure actibity → muscle breakdown → ↑ myoglobin.

PT p. 330

expanding intracranial mass → ↑ BP, ↓ HR

PT p. 331

• When the cord is injured traumatically, the initial findings are loss of deep tendon reflexes and flaccid weakness. UMN, such as hyperreflexia, spascticity, & Babinski sign, return after a few days to weeks.
• ↑ dose of steriods (at least 100 mg dexamethasone) is used for tx.

BP p. 156

• LMN abnormalities - anterior horn cell (α motor neurons) degeneration → weakness, fasciculations, and wasting ; amyotropic - loss of muscle mass.
• UMN - involvement of corticospinal tracts (lateral).
• ALS causes NO SENSORY DEFICITS or abnormal eye movements!
• Riluzole can retard progression of disease.

BP p. 157

• compression of sacral roots

BP p. 158

• venous sinus thrombosis
• this is very dangerous and needs to be excluded with venous sinus imaging.
• pseudotumor - commonly seen in women of childbearing age, or women on OCP or tetracycline.

BP p. 73, Kap m000912m

• postinfectious cerebellitis
• between ages 2-7
• usually follows varicella or other viral infxn.
• lasts a few weeks → complete recovery.

BP p. 59

• PML
• tx w/ HAART
• CT will show diffuse lesions of white matter.

Kaplan m000918

• lower extremity weakness and proximal muscle atrophy.
• CK and EMG are within normal limits.
• will slowly (weeks to months) improve with cessation of steroids.

Kap m000913, UW 4443

• electromyography under repetitive stimulation → progressive ↓ in amplitude of muscle potential.
• edrophonium challenge test is useful in dx MG, but is not completely specific.
• tx of myasthenic crisis: plasmapheresis or IV-Ig.

Kap Q0534m, Q1027m

• systemic vasculitides such as Churg-Strauss, SLE, PAN, cryoglobulinemia.
• other signs and symptoms include palpable purpura, arthralgias, hypocomplementemia.
• mononeuritis multiplex: painful asymmetric asynchronous sensory and motor peripheral neuropathy.

Kap m000541m

• small cell cancer of the lung.

Kap Q1029m

• 3-Hz spike and slow wave discharges.
• tx with ethosuximide (drug of choice), valproic acid, lamotrigine.

PT p. 35, Kap m000300m

• TCA (imipramine, desipramine, amitryptiline), gabapentin, pregabalin.
• acyclovir & prednisone to treat an acute episode of zoster and perhaps ↓ incidence of postherpetic neuralgia.

PT p. 85, Kap imq816

• commonly occurs unilaterally in an upper extremity after trauma to that extremity.
• hypothesized to be due to aberrant nerve healing.
• occurs in 3 phases: 1) severe pain from innocuous stimuli + redness, swelling, hyperhidrosis (sweaty); 2) similar pain + atrophy of skin and its appendages, coolness & pallor of affected appendage; 3) pain with continued atrophy of skin, underlying muscle, and flexion contracture → claw hand deformity.

Kap m000895m

• flaccid paralysis, loss of bowel & bladder fxn, loss of pain & temperature.
• ASA supplies anterior horn cells and part of the pain and temperature pathways.

Kap Q0631

• in contrast to intracerebral, intracerebellar bleeds/hematomas must be taken care of surgically emergently.
• patient with cerebellar hematomas remain lucid until cerebellar tonsillar herniation.

Kap Q0142

• results from vitamin B₁₂ deficiency.
• degeneration of posterior & lateral white matter tracts (dorsal columns & lateral corticospinal tracts).
• presents with insidious onset of parasthesias in hands & feet → weakness → spasticity.
• measure serum homocysteine & methylmalonic acid.

BP p. 128

• CT scan of head w/o contrast
• rule out life threatening processes such as intracranial hemorrhage.

UW 2671

• Staphylococcus aureus
• MRI of lumbosacral spine
• indications for imaging in patient with back pain: IV drug use, urinary catheter, steroid use, immunosuppression, osteoporosis, diabetes, age ≤ 18 or ≥ 50, hx of malignancy.

Kap m000919m, m000330m

• Spurling sign (↑ radicular pain by extension and lateral bending of neck toward side of lesion).
• Lhermitte sign (electrical shock with neck flexion).
• ↓ range of motion of neck.
• can cause HA
• osteophytes may impinge on cervical roots.
• paroxysmal vertigo with compression of vertebral artery → transient cerebellar ischemia.
• spondylosis: degenerative osteoarthritis of spinal vertebrae.

Kap Q0137m

• ↓ CSF absorption at arachnoid villi or obstructive hydrocephalus
• wet, wacky, and wobbly (urinary incontinence, dementia, abnormal gait).

UW 4651

• Definition: core temperature > 40 °C and AMS.
• tachycardia
• complications: rhabdomyolysis, renal failure, ARDS, coagulopathic bleeding (epistaxis).

UW 4703

serial measurements of bedside vital capacity.

VC < 15 ml/kg → ↑ risk for ventilatory failure.

UW 4271

• can indicate increased intracranial pressure.
• also cushing reflex (HTN & bradycardia)
• HA that is worse in morning.
• papilledema
• cranial nerve deficits
• somnolence
• confusion
• insteadiness

UW 4708

• familial hemiplegic migraine
• uncontrolled HTN
• CAD
• prinzemetal angina
• pregnancy
• ischemic stroke
• basilar migraine

UW 3620

• prominent gait, motor, or visual abnormalities.
• stepwise deterioration in mental function.
• typically patient's mental fxn will ↓ at a certain point, then some improvement, then infarct again.
• aka vascular dementia.

Kap m000294m2

• optic neuritis
• transverse myelitis (total loss of power, reflexes, and sensation).
• internuclear ophthalmoplegia
• paresthesias
• MRI findings of well-demarcated hyperintensity (characteristic periventricular distribution of white matter changes on T2 weighted images).
• must have MULTIPLE episodes!!!

Kap Q0160m

• neurocysticercosis
• CT shows calcified cysts (egg shells)
• viable cysts on CT are ring-enhanced.
• possible peripheral eosinophilia

Kap m000585m

• neurogenic claudication: pain radiating beyond back to buttocks, thighs, or legs.
• pain worse with extension of lumbar spine.

Kap m000572m

• Riluzole, a glutamate inhibitor
• side effects: dizziness, nausea, weight loss, ↑ liver enzyme, and skeletal weakness.

UW 3771

• botulism

UW 2289

• neuromuscular disorders
• think myasthenia gravis or botulism

UW 4464

• vasospasm → ischemia → infarction (tx w/ verapamil or other CCB).
• hyponatremia (inappropriate ADH secretion → H₂O retention → atria/brain natriuretic peptide → cerebral salt wasting).

UW 2678, 2284

• when pt w/ ischemic (thrombosis/embolism) stroke is not a candidate for tPA.
• 2° stroke prevention; however, hold ASA for 24 hours if tPA was administered.
• TIA (carotid atherosclerosis)
• prevention and tx of acute coronary syndromes (STEMI, NSTEMI, unstable angina).

UW4657, 3712

• multiple system atrophy
• when you see parkinsonism with autonomic symptoms (orthostatic hypotension, impotence, incontinence, etc.) think of this disease.
• anti-parkinsonism drugs are ineffective.
• tx with intravascular volume expansion (fludrocortisone, salt, alpha-adrencergic agonists, constrictive garments to lower body).

UW 4130

• thrombolytic is not contraindicated if pt is on ASA or clopidogrel.
• However, antiplatelet agents should not be used within the first 24 hrs after thrombolytic thx.
• contraindications: stroke/head trauma in past 3 mos, MI in past 3 mos, hx of intracranial hemorrhage, major surgery in pat 14 days, seizure at onset of stroke.

UW2288

• EEG; hyperventilation, photic stimuls, and sleep can trigger absence seizures.
• ethosuximide or valproic acid.

UW 2279

After MVA → complete weakness in both upper extremities, but able to move lower extremities.

What is the dx?

• central cord syndrome
• damage to the central portion of the anterior spinal cord, which contain the corticospinal tracts and decussating fibers in the lateral spinothalamic tract (component of the anterolateral spinal tract conducting pain & temp).
• commonly occurs with hyperextension injuries in elderly patients with degenerative changes in the C-spine.

UW3072

• emergent decompression (life-saving); otherwise → coma.
• SSx: ataxia, N&V, occipital HA, gaze palsy (eyes to one side), and facial weakness.
• No hemiparesis

UW3726