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Movement Disorders
Neurology
| Question | Answer |
|---|---|
| Action tremor: | Most common; arise when attempting to maintain a fixed position; frequency is 7-13 Hz; Essential or physiologic |
| Essential tremor = | resembles slower frequency physiologic tremor; essential or familial (auto dom); common in later life; extremities, head (titubation), or voice |
| Drug causes of physiologic tremor | Amphetamines, theophylline, lithium and valproate. |
| Essential tremor Tx | Firstline: BB: propranolol 60-240mg/day (CI in COPD; asthma; DM; bradycardia; AV conduction probs); Primidone 50/day - 125 TID (effective but sedating); Alprazolam for anxiety related; EtOH: temporary suppression |
| Ataxic (intention) tremor | Absent at rest/start of movement; dysmetria |
| Ataxic tremor: Causes: | Dz of cerebellum / connections (MS; tumors; infarct; ethanol-induced cerebellar degeneration) |
| Ataxic tremor: Tx: | Meds usu ineffective; Weights; surgical lesions of ventrolateral thalamus may be effective in severe cases |
| Athetosis: | slow writhing purposeless movements usu involving hands, tongue & face; usu in kids w/ cerebral palsy or result of kernicterus or hypoxia (poss later in life d/t hypoxia) |
| Chorea: Sx | Involuntary, irregular jerky movements; can cause continuous movements. |
| Chorea: may be due to: | untreated strep infxn (Sydenham chorea), PG (chorea gravidarum) or Huntington dz |
| Tics | onset 2-13 yrs; tx Haldol/pimozide |
| Hemiballismus | Violent flinging movements; Unilateral hemiballismus usu d/t infarct in contralateral subthalamic nucleus of Luys |
| Myoclonus = | Shock-like contraction of a group of mx; irreg in rhythm/amplitude; may involve restricted grp mx or be generalized. |
| Myoclonus may result from: | anoxic damage, spinal cord injury, uremia, hepatic encephalopathy or other rare neuro syndromes |
| Myoclonus Tx | Clonazepam, Valproate (Both have limited efficacy) |
| Dystonia: | Maintenance of a persistent extreme posture in one or more joints. |
| Generalized dystonia: | dystonia musculorum deformans; a rare hereditary dystonia |
| Focal dystonias: | torticollis (dystonia of the neck), writer's cramp & blepharospasm (dystonia of the mx’s involving eye closure). |
| Dystonia: Tx | Botox injxn increasingly common & helpful; Surgical tx (torticollis) also available; Medical tx (benzos, anticholinergics, neuroleptics) generally unsatisfactory |
| Parkinson dz: epidemiology | 500,000 in US; onset 40-70 yo; M=F |
| Parkinson dz: Sx | Unilateral onset. Rest tremor/3-6 hz; pill rolling; cogwheeling; rigidity; bradykinesia; difficulty initiating movement. Masked facies; stooped posture, shuffling or festinating gait. Diminished postural reflexes & eye blinking rate. Micrographia |
| Parkinson dz: Pathology | Degeneration & loss of pigmented cells in nigrostriatal pathway, raphe nuclei, locus ceruleus & motor nucleus of vagus; dramatic loss of DA-containing neurons |
| L-DOPA | 80% of pts improve; Sinemet treats akinesia & is less effective in treating tremor. |
| L-DOPA MOA | precursor of DA (which cannot cross BBB; L-DOPA can) |
| Dopamine agonists (Parlodel, Mirapex, Requip) MOA | Act like DA; direct DA effect on striatal neurons; may allow for reduction in dose of Sinemet required & may decrease on-off phenomena |
| Amantidine MOA | May increase DA release from presynaptic storage site at nerve terminals; weakly effective |
| Anticholinergic drugs (Artane, Cogentin) MOA: | May be more effective for tx of tremor; AE = confusion, constipation, dry mouth, urinary retention |
| Selegiline MOA | inhibits monoamine oxidase type B |
| Cabergoline MOA | inhibits COMT |
| Huntington Dz presentation | AD, onset 30-50 yo; Sx often begin w/ psych disorder (immaturity, impulsivity, depression); later apathy & dementia; Chorea, Athetosis; butterfly ventricles (caudate atrophy) |
| Huntington chorea due to: | Decreased activity of acetylcholine & GABA neurons; increased activity of dopaminergic neurons |
| Huntington Tx | For sx reduction only. Tetrabenazine or amantadine 25 TID for chorea. DA receptor blockers (haloperidol) for behaviors/dyskinesias; clozapine |
| Wilson dz | Auto rec; onset 2nd-3rd decades; first sx: hepatic dysfn/cirrhosis; tremor/ bradykinesia, dysarthria, dysphagia; limbs rigid, facial mx fixed empty smile; arms: wing-beating tremor; Kayser-Fleischer (deep cornea) |
| Wilson: labs | Serum ceruloplasmin (Cu carrying pro) very low; urine Cu high |
| Wilson: patho logy | CNS changes: brownish discoloration of some basal ganglia; proliferation of protoplasmic astrocytes (Alzheimer Type II cells) |
| Wilson: Tx | Should begin before neuro Sx onset; early tx prevents neuro sequela devt; eat low Cu foods; sulfurated potash with meals (prevent Cu absorption); chelator (d-penicillamine) to remove absorbed Cu |
| Wilson dz: Tx: foods to avoid | liver; chocolate; mushrooms; shellfish; nuts |
| Tardive dyskinesia: cause | Iatrogenic d/t LT neuroleptic tx (esp Haldol; also atypicals, poss metoclopramide, amphetamines, L-dopa); may be result of DA receptor supersensitivity |
| Tardive dyskinesia: Sx | Abnormal BLM (tongue thrusting & chewing); head movements (turning & bobbing); poss also abnormal limb movements; Sx can fluctuate & may take mos-yrs to resolve (sometimes permanent) |
| Tardive dyskinesia: Tx | stop the offending drug; many tx tried w/ marginal success |
| Tardive dyskinesia: most successful tx: | achieved with DA-depleting agents (tetrabenazine); also Vitamin E? |
| Excess DA results in: | dyskinesia and chorea |
| Dopamine deficiencies cause: | Parkinsonian-like symptoms |
| Action of acetylcholine & DA: | Act in opposite directions; adding DA is equivalent to blocking acetylcholine |
| CJD Sx | usu later in life; rapidly progressive dementia, myoclonus, ataxia & somnolence; epileptiform pattern on EEG |
| MS epidemiology | F>M; 350K in US (1M world); onset 20-40 yo; most present w/relapsing form; prevalence increases with distance from equator |
| MS S/S | Optic (retrobulbar) neuritis; Transverse myelitis; Paresthesias; Ataxia; Weakness or incoordination; Spasticity; Cognitive impairment |
| MS: MRI findings | multiple characteristic lesions or plaques: periventricular or subcortical U-fibers, corpus callosum lesions |
| MS: CSF findings | evidence of oligoclonal bands or increased IgG index |
| MS: types of dx criteria | Schumacher; Poser: Macdonald |
| MS: Goals of tx | Tx whole dz; slow accumulation of disability; Reduce relapse rate & CNS inflam (lesions) & progression of brain atrophy (shrinkage); improve pt QOL (including cognitive) |
| MS Tx options | Beta-interferon; SQ glatiramer; poss immunosupp adjuncts for aggressive/ breakthrough; corticosteroids (methylprednisolone) for relapse |
| Forms of MS: | relapsing-remitting, primary progressive, secondary progressive |
| Tourette etiology / pathology | Auto dominent; can be presentation of Wilson dz. Onset usually 2-15 yo |
| Recurrent episodes of vision change, diplopia, weakness & tingling in extremities that resolve | MS |
| CJD S/S | usu later in life; rapidly progressive dementia, myoclonus, ataxia & somnolence; epileptiform pattern on EEG |
| MS S/S | Optic neuritis; Transverse myelitis; Paresthesias; focal neuralgia; Ataxia; Weakness/ incoordination; Spasticity; Cognitive impairment |
| Lhermitte’s sign = | electrical sensation down body w/ neck flexion; seen in MS |
| MS pathophys | immuno d/o assoc w/CNS Ig prodn & T-lymph alteration; poss viral etio |
| MS pattern of sxs | affect multiple areas over time (if they don't, prob not MS) |
| Ataxic (intention) tremor | Absent at rest/start of movement; dysmetria; Dz of cerebellum / connections (MS; tumors; infarct; ethanol-induced cerebellar degeneration) |
| Dystonia: | Maintenance of a persistent extreme posture in one or more joints. |
| Focal dystonias: | torticollis, writer's cramp, blepharospasm; tx Botox / surg |
| Huntington Dz presentation | AD, varying age of onset; Sx often begin w/ psych disorder (immaturity, impulsivity, depression); later apathy & dementia; Chorea, Athetosis; butterfly ventricles (caudate atrophy) |
| Huntington chorea due to: | DA excess state |
| Wilson dz | Auto rec; onset 2nd-3rd decades; first sx: hepatic dysfn/cirrhosis; tremor/ bradykinesia, dysarthria, dysphagia; limbs rigid, facial mx fixed empty smile; arms: wing-beating tremor; Kayser-Fleischer (deep cornea) |
| Wilson: patho | CNS changes: brownish discoloration of some basal ganglia; proliferation of protoplasmic astrocytes (Alzheimer Type II cells) |
| Tardive dyskinesia: Sx | Abnormal BLM (tongue thrusting & chewing); head movements (turning & bobbing); poss also abnormal limb movements; Sx can fluctuate & may take mos-yrs to resolve (sometimes permanent) |
| Tardive dyskinesia: most successful tx: | achieved with DA-depleting agents (tetrabenazine); also Vitamin E? |
| Action of acetylcholine & DA: | Act in opposite directions; adding DA is equivalent to blocking acetylcholine |
| RLS can be primary, or secondary to: | periph neuropathy, uremia, PG, Fe def |
| ALS: Dx criteria | UMN & LMN sx in 3 regions; UMN: clinical; LMN: EMG; CK nml or high |
| Primary lateral sclerosis: path | degeneration of lateral corticospinal tract |
| Primary lateral sclerosis clin findings | usu legs before arms; leg weak/stiff; spasticity; ave duration >8 yrs; many pts develop LMN sx & transition to ALS |
| progressive muscular atrophy | pure LMN dz; slower progress than ALS; often spares bulbar mx; CK very high |
| Wilson manifestations: | hepatolenticular degen (impaired ceruloplasmin synth); presents in teen years (hepatitis); tremor, dysarthria, slow, hoarse, chorea |
| Parkinson: TRAP = | Tremor, Rigidity, Akinesia, Postural instability |
| Most common genetic form of Parkinson: | PARK8 |
| Progressive demyelinating; brain, sp cord, optic n.; viral; F>M, peak 20-40yo | MS |
| Entacapone is tx for: | Parkinson dz |
| Entacapone side effect | brown urine |
| Damaged areas in ALS | corticospinal tracts, anterior horn cells, bulbar motor nuclei |
| MS testing | MRI, VER, BAER, SSEP, CSF oligoclonal banding, spinal fluid IgG |
| MS S/S | 15-50 yo; optic neuritis; fatigue; Lhermitte sx; Uhthoff's phenomenon |
| MS tx | methylprednisolone/ IVIg for acute; glatiramer & interferon for relapsing- remitting sx |
Created by:
Abarnard
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