*ONCE GET PROTEINURIA DAMAGE IS IRREVERSIBLE
1. AUTOSOMAL DOMINANT - -associated with OCD
2. multiple motor tics - blinking, grimacing, head jerking, shoulder shrugging
phonic tics (>1 kind)- grunting, sniffing, clearing throat, coprolalia
3. clonidine, pimozide, haloperidol
854.dementia 1. diagnostic criteria 2. vascular dementia 3. binswanger's disease
1. progressive loss of intellectual function, with PRESERVATION OF CONSCIOUSNESS, associated with inc.
age
2. stepwise deterioration in intellectual function from cerebral infarction 3. diffuse subcortical white matter degeneration in patients with atherosclerosis/HTN
855.primary causes of dementia
alzheimer's disease, vascular dementia, binswanger's disease, brain tumors, chronic subdural hematomas, NPH, lewy body dementia, picks disease, MS, parkinsons, huntington, wilsons
856.infectious causes of dementia
HIV infection (AIDS dementia), neurosyphilis, cryptococcus, CJD, progressive multifocal
leukoencephalopathy
857.metabolic causes of dementia
thyroid disease, B12 deficiency, thiamine deficiency (--> korsakoff dementia), niacin deficiency
858.what toxins cause dementia
chronic alcoholism, aniline dyes, lead, mercury
859.pseudodementia severe depression that can cause a decline in cognition but is responsive to antidepressants
patients are overly concerened about their memory loss
860.1. dementia lab workup 2. dementia pharmacologic treatment
1. CBC with diff, chemistry panel, TSH, B12, folate, VDRL, HIV screening, CT or MRI of head
2. vitamin E, tacrine, donepezil
861.alzheimers disease 1. risk factors 2. pathology findings 3. dx/tx
1. age : 10-15% of people over age 65, 15-30% over age 80
family history, downs syndrome, chromosome 21, 14, 19 abnormalities, loss of CHOLINERGIC neurons*
2. senile plaques- dilated, tortuous neuritic plaques around beta amyloid core, neurofibrillary tangles - in cytoplasm of neurons
3. clinical diagnosis, CT or MRI shows diffuse cortical atrophy with
ventriculomegaly
tx = cholinesterase inhibitors - donepezil, rivastigmine, galantamine
memantine - NMDA antagonist ginkgo, lecithin
vitamin E 2000IU/day
HRT associated with lower risk of alzheimers disease
862.alzheimers disease 1. clinical pres
1. early - forgetfulness, impaired ability to learn, poor performance, change in judgement
intermediate - progressive memory impairment, visuospatial disturbances later stages- help needed for daily living, difficulty remembering names of relatives advanced- complete debilitation, loss of bowel/bladder control,
death 2/2 infection
863.lewy body dementia 1. clinical pres 2. tx
1. visual hallucinations predominant initially, disorganized speech, extrapyramidal symptoms, fluctuating mental status, faster progression 2. selegiline, neuroleptic agents (for hallucinations/psychosis)
864.altered mental status 1. what part of the brain is responsible for arousal 2. what part of the brain is responsible for cognition
1. RAC in brainstem 2. cerebral cortex
865.causes of delirium
P.DIMMWIT
postoperative, dehydration/malnutrition, infection, medications, metals, withdrawal stages, inflammation/fever, trauma/burns
866.what drugs can cause delirium
TCAs, corticosteroids, anticholinergics, hallucinogens, cocaine
867.delirium 1. clinical pres 2. dx/tx
1. rapid deterioration of mental status, FLUCTUATING LEVEL OF
CONSCIOUSNESS, disorientation, frequently abnormal vital signs, may include
hallucinations
2. MMSE, chemistry panel, B12, thiamine tx = underlying cause, haloperidol for agitation, supportive
868.ddx for coma/stupor
SMASHED
structural brain pathology - stroke, subdural/epidural, tumor, abscess, herniation
meningitis, mental illness alcohol, acidosis
seizures, substrate deficiency (thiamine) hypercapnia, hyperglycemia, hyperthermia, hyponatremia hypoglycemia, hypoxia endocrine - addisonian crisis, thyrotoxicosis, hypothyroidism, encephalitis (uremia) drugs - opiates, barbiturates, benzos, dangerous compounds - monoxide, cyanide, methanol
869.coma 1. criteria 2. causes 3. diagnosis
1. decreased LOC, patient unresponsive to any stimuli
2. bilateral structural brain lesions, or brainstem, or cerebellum, or RAC damage, global brain dysfunction - metabolic or systemic disorders
psychiatric - conversion, malingering 3. ABC, assume trauma, stabilize cervical spine, assess LOC (via GCS)
asymmetric movement = most likely mass lesion
pupillary light reflex - indicates midbrain intact, morphine, anoxic encephalopathy, bilateral fixed/dilated (severe anoxia), unilateral fixed /dilated uncla herniation, pinpoint pupils - ICH, narcotics independent breathing - intact brainstem toxicologic analysis
CT or MRI of brain
LP if suspect meningitis or SAH
870.coma 1.
treatment 2 brain death 3.
persistent vegetative state
1. correct cause: airway, supp. o2, naloxone, dextrose, thiamine before glucose, BP, electrolytes, lower ICP if herniation 2. irreversible absence of brain/brainstem function, no brainstem reflexes (pupils, calorics, gag, cornea, dolls eyes)
no drug intox or metabolic condition core body temp >32 C
clinical evidence or CT/MRI shows causative explanation for brain death
repeat examinations, or EEG shows isoelectric activity
3. comatose, but eyes are open, appear awake, may have random limb/head movements, spontaneous respirations
871.multiple sclerosis 1. patho 2. clinical pres
1. selective CNS white matter demyelination of the brain and spinal cord, classically at the angles of the ventricles, pyramidal, cerebellar pathways, MLF, optic nerve, posterior columns 2. transient sensory deficits, paresthesias/dec.
sensation of upper/lower limbs, fatigue*, weakness/spasticity, leg stiffness, optic neuritis, central scotoma, dec. pupillary reaction, INTERNUCLEAR OPHTHALMOPLEGIA (adduction deficit), bilateral trigeminal neuralgia
cerebellar involvement- ataxia, intention tremor, dysarthria,
incontinence, impotence, constipation memory loss, personality change, emotional lability, anxiety, depression
neuropathic pain/hypersesthesas
872.multiple sclerosis 1. dx
1. clinically definite - two episodes, evidence of white matter lesions clinical or imaging laboratory supported - two episodes of
symptoms, at least one white matter MRI lesion, abnormal CSF
probable MS- two episodes of symptoms, and either one white matter lesion, or oligoclonal bands
evoked potentials - remyelinated neurons conduct slowly
873.multiple sclerosis 1. clinical course
clinically silent - stable or benign disease relapsing and remitting -
primary progressive - onset typically later 1/3 of patients develop debilitating disease
874.multiple sclerosis 1. tx
1. high dose IV methylprednisone for acute attack
plasma exchange therapy for steroid refractory acute attacks
DMT- interferon B1a, B1b, glatiramer acetate reduce relapse rates by 35% (SE is flu like symptoms)
cyclophosphamide for rapidly progressing disease
baclofen or dantrolene for spasticity carbamazepine or gabapentin for neuropathic pain
875.locked in syndrome 1. clinical pres 2. patho
1. patients completely paralyzed except for respiration, blinking, and vertical eye movement, patients are fully aware of their surroundings and can feel pain
2. hemorrhage or infarction of the ventral pons
876.guillain barre syndrome 1. patho 2. clinical pres
1. mycoplasma, campylobacter jejuni, CMV, hepatitis, HIV, hodgkins disease, lupus, HIV seroconversion --> inflammatory
demyelinating polyneuropathy of peripheral nerves
2. abrupt onset of ascending symmetric weakness/paralysis of all four extremities, PRESERVED SENSORY* respiratory, facial, bulbar muscles, spares sphincter
control/mentation
autonomic features - arrhythmias, tachycardia
877.guillain barre 1. dx/tx
1. **CSF shows elevated protein**, normal cell count, dec. NCV on EMG
tx= pulmonary monitoring/mechanical ventilation, IVIG, plasmapheresis, DO NOT GIVE STEROIDS IN GUILLAIN BARRE SYNDROME
recovery usually occurs in 1-3 weeks, but if persists >6 weeks probably will have relapsing course
878.myasthenia gravis 1. patho 2. clinical pres 3.
myasthenic crisis + tx
1. autoimmune disorder against nicotinic Ach-R, results in muscle fatigue especially rapidly stimulated muscles
2. skeletal muscle weakness exacerbated by use, involves cranial nerves
first symptoms often diplopia, ptosis, blurred vision, dysarthria, dysphagia
3. infection or stressor -->
diaphragm/intercostal fatigue results in respiratory failure
ET tube + withdrawl anticholinesterases + plasmapheresis
879.myasthenia gravis 1. dx
1.anti- Ach-R test
EMG shows decremental response CT scan of thorax to rule out thymoma (10% of patients 75%
histologically abnormal) edrophonium tensilon test tx = pyridostigmine,
thymectomy - can cause complete remission
3rd line agents: corticosteroids, azathioprine, cyclosporine IVIG for exacerbations
serial FVCs <15 ml/kg = indication for intubation
880.duchenne's muscular dystrophy 1. patho 2. clinical pres 3. dx/tx
1. X LINKED RECESSIVE - mutation in dystrophin causes muscle fibers to die and replaced by fibrous scar tissue 2. progressive, symmetric proximal muscle weakness, especially the pelvic girdle
Gowers maneuver (proximal lower extremity weakness),
pseudohypertrophy (fat replaces muscle), wheelchair confinement, respiratory failure/death by third decade.
3. serum CK, muscle biopsy, DNA testing
881.Becker's muscular dystrophy
1. x linked recessive, similar to duchennes muscular dystrophy but less severe, some dystrophin present
882.what drugs exacerbate myasthenia gravis
aminoglycosides, tetracyclines beta blockers
quinidine, procainamide, lidocaine
883.neurofibromatosis type 1
1. patho/clinical