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Movement Disorders
| @NeuroFOD | |
|---|---|
| Movement Disorders | Movement Disorders |
| Movement Disorders | |
| Parkinsons histopathology | alpha-synuclein, +ubiquitin |
| Influence of indirect vs direct pathway on movement | Indirect = inhibits movement. Direct = increases movement. Dopamine = \/indirect /\direct |
| Parkinsons neuroanatomical structure | Substantia nigra pars compacta. Not pars reticulata |
| Two sections of the substantia nigra | Compacta = dopaminergic = Parkinsons. Reticulata = GABA = may have role in epilepsy |
| Common secondary causes of Parkinsons disease | Wilsons, lacunar infarcts (hemiparkinsons), carbon monoxide, manganese, methotrexate, Fahr |
| Parkinsons genes | PARK1 = alpha-synuclein. PARK2 = parkin. DJI. LRRK2 = late-onset in Ashkanazi jewish |
| Parkinsons gene for late onset Parkinsons | LRRK2 = late-onset in Ashkanazi jewish |
| Parkinsons symptoms | TRAP = tremor (3-7Hz), rigidity, akinesia, postural instability |
| Parkinsons common exam findings | Olfaction, Myerson's blink, blepharoclonus, hypomimia |
| Hypomimia definition | Cannot do spontaenous or posed expressions and can't recognize expressions. A/w Parkinsons |
| 1o vs 2o Parkinsons symptom | Olfaction is affected in primary Parkinsons, but generally preserved secondary |
| 1o vs 2o Parkinsons DAT scan findings | 1o = asymmetric basal ganglia degeneration. 2o = symmetric |
| Parkinsons PET scan findings | Reduced occipital metabolism |
| What molecule is used for Parkinsons DAT scan? | Tissue tagged with Dopamine Decarboxylase. 1o = asymmetric degeneration, 2o = symmetric |
| Lewy Body vs Parkinsons clinical feature | LBD = 1st dementia, then movement after ~1yr. Also more neuropsych (hallu, REM disorder) |
| Hallucinations of Lewy Body vs Schizophrenia | Not a 100%, but generally LBD has visual hallucinations whereas Schizo has auditory |
| Most common etiology of hemiparkinsonism | Vascular - either lacunar infarct or hypertensive hemorrhage. Then corticobasal degeneration |
| Lewy Body histopathology | Intracyoplasmic eiosinophils with nigral pallor. Very commonly asked -- know terms & image |
| Term for substantia nigra histopath finding in Parkinsons | Marinesco body |
| 1st line treatment of Parkinsons | Levodopa. Note this is a precursor and will be metabolized to the active dopamine form |
| What to do if Levodopa causes GI sideeffects? | Add carbidopa = reduce peripheral conversion of levodopa to active dopamine |
| What to do if Levodopa causes end-of-dose wearing off? | Add entocapone = COMT-inhibitor = gives more sustained pharmacokinetics for levodopa |
| What to do if Levodopa causes dyskinesias? | Add amantadine. 2nd choice is clozapine |
| What do do if Levodopa causes unpredictablity? | Add Apomorphine or inhaled dopamine |
| Side effect of amantadine | Hallucinations |
| Parkinsons med that causes hallucinations | Amantadine. More so than acetylcholinesterase inhibitors or levodopa |
| Parkinsons / LBD -- memory loss treatment | Rivastigmine is the ONLY FDA approved. Other ACEi = donepezil. Avoid memantine |
| Parkinsons / LBD -- incontinence treatment | Avoid acetylcholine inhibitors like oxybutynin. Use mirabegron (beta-3 agonist) |
| Parkinsons / LBD -- psychosis treatment | Primavenserin = inverse agonist at seretonin 2-HA receptors |
| Most common side effect of levodopa | Constipation |
| Lewy Body disease associated with sleep disoder | REM sleep behavior disorder |
| Meds to avoid in Lewy Body disease | Avoid antipsychotics (haldol, etc), antihistamines, anticholinergics. Of all, least bad = quetiapine |
| Posterior cortical atrophy | Isolated occipital hypometabolism (without any Parkinsons symptoms) |
| Parkinsons med that causes impulse control disorder | All gambling/sex/etc = blame Pramiprexole. Much higher than amatadine or levodopa |
| MSA vs Parkinsons | MSA = must have significant dysautonomia. Parkinsons can as well, but less severe |
| MSA histopathology | alpha-synuclein. Will show glial cytoplasmic inclusions |
| MSA-A (Shy-Drager) | Max autonomic dysfunction (incontinence, annhydrosis, erectile dysfunction, etc) |
| MSA-P (substantionigral) | Max Parkinsons symtoms (TRAP). MRI shows iron deposition in substantia nigra |
| MSA-C (olivopontocerebellar) | Max ataxia. They bend laterally Pisa tower. MRI shows classic hot cross bun sign |
| MSA-C MRI finding | Hot cross bun sign. Due to degradation of the olivopontocerebellar tracts in the midbrain |
| Hot cross bun sign neuroanatomy | MSA-C. Due to degradation of the olivopontocerebellar tracts in the midbrain |
| MSA treatment | Try levodopa, but classically poor response. Try fludrocortisone/midodrine for autonomic |
| MSA -- autonomic symptoms treatment | Fludrocortisone or midodrine |
| PSP histopathology | tau. Tufted astrocytes with globose neurofibrillary TAU tangles inclusions. NOT alpha-synuclein |
| PSP MRI finding | Hummingbird beak sign. Due to midbrain and superior peduncle atrophy |
| Hummingbird beak sign neuroanatomy | PSP. Due to midbrain and superior peduncle atrophy |
| PSP main symptoms | Up-gaze palsy = move head up to see higher = fall backwards |
| PSP vs Parkinsons posture differences | Parkinsons = kyphosis. PSP is opposite (can't look up = move body/face up = hyperextension) |
| PSP PET findings | Hypometabolism in basal ganglia + thalamus + anterior cingulate |
| PSP treatment | Try levodopa, but classically poor response |
| Tufted astrocytes with tau inclusions | PSP |
| Corticobasal degeneration symptoms | uL hemiparkinsonism, uL hand apraxia (alien limb), uL sensory loss, +myoclonus, +Balint syndr |
| Corticobasal degeneration MRI | CBD = one-sided hemisphere degeneration. It is degenerative, not inflammatory |
| Corticobasal degeneration histopathology | tau. Tau plaques like Alzheimers (not tangles like PSP). Spongy balloon cells |
| Alien limb | Hand apraxia (involuntary, semi-purposeful movements that worsen with eye closure). A/w CBD |
| Corticobasal degeneration syndrome association | A/w Balint syndrome |
| Stiff person syndrome antibodies | Anti-GAD65 (glutamic acid decarboxylase), Anti-Amiphiphysin, Anti-Ri |
| Stiff person syndrome pathophysiology | Glutamic acid decarboxylase def = glutamate can't convert to GABA = \/GABA = spasticity |
| Rigidity, ataxia, pain. Anti-GAD65+ | Stiff person syndrome |
| Stiff person syndrome treatment | Immunosuppression (IVIG/steroids) |
| Differentiate tetanus from stiff person syndrome | Both have rigidity and spasticity. Tetanus will have laryngospasm, but stiff person will not |
| Tremor -- worse with SSRI | Enhanced physiologic tremor. Worsens with SSRIs and caffeine |
| Tremor -- 4-12Hz, postural+action, worse w/ intention | Essential tremor |
| Essential tremor neuroanatomy | Cerebellar and inferior olive |
| Essential tremor vs Parkinsons tremor | Essential = asymmetric, in action. Parkinsons tremor = symmetric, worse with rest |
| Essential tremor treatment | Propanolol IF no asthma/cardiac/diabetes. Otherwise primidone. Benzos/botox/DBS 2nd line |
| Primidone used for? | Essential tremor. 1st line with propanolol |
| Tremor -- worse with standing, better with walking | Orthostatic tremor |
| Tremor -- worse with distraction, better w/ chin touch | Dystonic tremor. Chin touch is the sensory trick patient's discover to lessen tremor |
| Dystonic tremor treatment | Botox is 1st line |
| Tremor -- rest+postural+action, clicking sound, wing-beat | Dentatorubroolivary tremor. Clicking sound is from palatal myoclonus |
| Dentatorubroolivary tremor neuroanatomy | Central tegmental tract of the Guillian Mollaret triangle |
| Guillian Mollaret triangle | iL red nucleus, iL olivary nucleus, cL dentate nucleus. Central tegmental tract b/t olivary-dentate |
| Tremor -- better with distraction, variable amplitude | Psychogenic tremor |
| Psychogenic tremor treatment | Clonazepam b/c likely d/t anxiety. Can SSRIs |
| Dystonia definition | Slow sustained contraction of both antagonist and agonist muscles. Leads to twisted postures |
| Dystonia -- neck/limbs | Cervical dystonia |
| Cervical dystonia treatment | Botox is 1st line. Can acetylcholine antagonists (trihexyphenidyl). Can cervical rhizotomy |
| Dystonia -- bilateral repetitive blinking | Blepharospasm = cranial dystonia. Of the orbicularis oculi muscles |
| All dystonia 1st line treatment | Botox |
| Dystonia -- bilateral repetitive blinking + hemifacial spasm | Meige syndrome |
| Eessential vs myoclonic dystonia | Essential = face/neck only. Myoclonic dystonia = affects face/neck + limbs |
| Persistent cortical myoclonus + ataxia | Myoclonic Ataxia = Ramsey Hunt syndrome (the other one, not the CN7 palsy) |
| Post-hypoxic (cardiac arrest) myoclonus | Lance-Adams syndrome. 9% can good outcomes. ONLY if myoclonic status = sure poor progn |
| Cortical myoclonus + rapid dementia | Creutzfeldt-Jakob Disease |
| Sudden shock-like movements that worsen w/ stimulation | Myoclonus |
| Myoclonus treatment | Valproate, Clonazapam. Then Keppra or SSRI |
| Meds that can worsen myoclonus | CBZ, phenytoin, other sodium-channel AEDs |
| Startle myoclonus | Hyperekplekia. CJD, Stiff person syndrome, MS. In child = hereditary hyperekplekia |
| Hemifacial spasm pathophysiology | Peripheral myoclonus d/t compression of CN7 |
| Hemifacial spasm treatment | Botox is 1st. Then baclofen, carbamazepine, surgery |
| Huntingtons disease gene | CAG repeats in Huntingtin gene on Chromosome 4p. Inheritance is autosomal dominant |
| Huntingtons disease inheritance | Autosomal dominant |
| Huntingtons disease pathophysiology | Huntingtin gene functions in axonal transport. \/transport = /\calcium excitotoxicity |
| Will Huntingtons patients suffer cognitive decline? | Yes. Huntingtons is more than just chorea. Will have cognitive decline + hallucinations/psychosis |
| Irregular, arrythmic, involuntary, rapid movements | Chorea |
| Milkmaid grip | Motor impersistence. During handshake, pt squeezes but can't hold on (looks like milking a cow) |
| Huntingtons MRI | Basal ganglia atrophy |
| Huntingtons genetic testing | No one under 18. Must undergo genetic counseling prior to testing. All b/c there's no cure |
| Huntingtons inheritance | Autosomal dominant. However, varialble penetrance depending on # of CAG repeats |
| Huntingtons treatment | Dopamine depleters = tetrabenazine + reserpine. Can also VPA, clonazepam, anti-psychotics |
| Huntingtons-like + progressive myoclonic epilepsy | Dentatorubropallidoluysian Atrophy. Also CAG repeats but in Atrophin-1 gene |
| Dentatorubropallidolysian atrophy MRI | Basal ganglia atrophy like Huntingtons + cerebellum/brainstem atrophy |
| Huntingtons-like + orofacial/lingual dystonias | Neuroacanthocytosis |
| Neuroacanthocytosis histopathology | Acanthocytes = spiculated RBCs |
| Neuroacanthocytosis treatment | It is associated with abetalipoproteinemia. So Vitamin E may help |
| Huntingtons-like + cranial/limb dystonias + motor deficits | Pantothene Kinase Associated Neurodegneration PKAN |
| PKAN gene | PANK2 |
| PKAN inheritance | Autosomal recessive. Opposed to autosomal dominant of Huntingtons |
| PKAN MRI | Eye of the tiger = iron deposition in substantia nigra reticulata and globus pallidus |
| PKAN treatment | No benefit from iron chelation therapy |
| PKAN vs Wilsons disease | Similar symptoms, but PKAN never liver failure whereas Wilsons must have atleast transaminitis |
| Sydenhams chorea pathophysiology | IgG to Grp-A beta-hemolytic Strep |
| Friedrichs ataxia gene | GAA in Frataxin on chromosome 9q13. \/mitochondria |
| Ataxia, propriocep, LE weak/spasticity, areflex, dysarthria | Friedrichs ataxia |
| Ataxia + HOCM or AV block or A.fib | Friedrichs ataxia |
| Ataxia + pes cavus | Friedrichs ataxia |
| Friedrichs ataxia inheritance | Autosomal recessive |
| Friedrichs ataxia associated symptoms | Pes cavus, scoliosis, HOCM/heartblock/A.Fib, DM, optic atrophy |
| Friedrichs ataxia vs SCA | SCA = no sensory symptoms and /\ DTRs. Friedrichs = vibr/proproception + areflexia |
| Friedrichs ataxia MRI | C-spine degeneration, but intact cerebellum |
| Friedrichs ataxia treatment | Try supplements -- CoQ10, Vitamin E, Iron chelators |
| How to people die in Friedrichs ataxia? | Cardiac arrythmias or HOCM |
| Ataxia Telangiectasia genetics | 7:14 translocation on ATM gene on chromosome 11 = a-tocopherol mutation = \/ vitamin E |
| Ataxia Telangiectasia inheritance | Autosomal recessive |
| Ataxia Telangiectasia pathophysiology | ATM gene = no a-tocopherol = \/ vitamin E = impaired dsDNA repair |
| Labs \/ vitamin E, \/ all Ig, /\ aFP | Ataxia Telangiectasia |
| Ataxia Telangiectasia MRI | Cerebellar and inferior olive atrophy |
| How do people die in Ataxia Telangiectasia? | Intracranial hemorrhages d/t rupture of telangiectasias in the brain |
| Ataxia Telangiectasia associated syndromes | Immunodeficiency, cancers. ICH d/t brain telangiectasias. Vision loss d/t conjunctival telang |
| Abetalipoproteinemia gene | MTTP on chromosome 4q24 |
| Ataxia + sensory loss + malabsorbtion syndrome | Abetalipoproteinemia |
| Abetalipoproteinemia labs | Severe \/ hgb, \/ cholesterol (especially LDL), \/ vitamins ADEK , \/ apolipoproteinB |
| Abetalipoproteinemia treatment | Vitamin E |
| Abetalipoproteinemia histopathology | Acanthocytes = spiculated RBCs. A/w neuroacanthocytosis |
| Phytanic acid | Refsum disease |
| Refsum disease protein | /\ phytanic acid |
| Ataxia + night blindness, neuropathy, deafness | Refsum disease. Has a relapsing-remitting course |
| Cerebrotendinous Xanthomatosis gene | 27-hydroxy bile acid synthesis defect = /\ cholesterol deposits |
| Ataxia + tendon xanthomas | Cerebrotendinous Xanthomatosis |
| Cerebrotendinous Xanthomatosis treatment | Colic acid (bile), simvastatin |
| Foamy astrocytes w/ diffuse demyelination | Vanishing white-matter leukoencephalopathy |
| Ataxia + seizures, episodic, post-fever/illness | Vanishing white-matter leukoencephalopathy |
| Behr disease | Ataxia, optic atrophy, nystagmus, neuropathy |
| Spinocerebellar ataxia inheritance | Autosomal dominant |
| Ataxia + no sensory symptoms + /\ DTRs | Spinocerebellar ataxia |
| SCA types | All ataxia + weakness. SCA 1,4,8,12 = opthalmoplegia, 2=areflexia, 3=Parkinsons, 7=visual loss |
| SCA gene | Ataxin. SCA-7 = CAG repeats on Ataxin-7 |
| SCA MRI | Progressive cerebellar atrophy |
| Juvenile Huntington Disease | CAG in Huntingtin (same as adult-onset). P/w ataxia + parkinsonism +seizures |
| Ataxia + parkinsonism, seizures, in chid | Juvenile Huntington Disease |
| Anti-ASO | Sydenhams chorea |
| Anti-DNA-b | Sydenhams chorea |
| Sydenhams chorea antibodies | IgG to Grp-A beta-hemolytic Strep, Anti-ASO, Anti-DNA-b |
| Sydenhams chorea treatment | Fluphenazine (DA-antagonist). + daily penicillin till 21yo to prevent rheumatic fever |
| Sydenhams chorea prognosis | Self-resolves <1yr. But must daily penicillin till 21yo to prevent rheumatic fever |
| Kernicterus | /\ unconjugated bili = hemolysis = chorea + deaf + impaired up-gaze |
| DYT1 genetics | Primary torsion dystonia. Autosomal dominant GAG in TOR1A Torsin gene |
| Child gait dystonia -> generalized w/in 5 yrs | Primary torsion dystonia. DYT1 |
| DYT5 genetics | Dopa-responsive dystonia. Autosomal dominant GTP-ase |
| Child dystonia -> parkinsons, worse PM, improv w/ sleep | Dopa-repsonsive dystonia. DYT5 |
| Dopa-responsive dystonia labs | DYT5. \/ homovanillic acid, \/ 5-HILA, \/ Biopterin |
| DYT5 treatment | Dopa-responsive dystonia. Give levodopa |
| Child dystonia 1st line treatment | Attempt levodopa trial. Works for DYT5 dopa-responsive dystonia, and also some others |
| Child dystonia + myoclonus, better with alcohol/benzos | Myoclonic dystonia. DYT11. Autosomal dominant SGCE e-sarcoglycan |
| e-sarcoglycan | Myoclonic dystonia. DYT11 |
| Tourette disorder criteria | MUST <18yo and >1yr of symptoms. MUST motor+vocal tics |
| Tourette disorder meds to avoid | Methylphenidate/stimulants. Often prescribed for comorbid ADHD but these can worsen tics |
| Tourette disorder associations | ADHD, OCD, bipolar |
| Tourette disorder treatment | alpha-2 agonists (guanfacine, clonidine). Can TPX, Keppra. Antipsychotics are 2nd line |
| Child dancing eyes + myoclonus + ataxia | Opusclonus-Myoclonus-Ataxia syndrome |
| Opusclonus-myoclonus-ataxia syndrome treatment | Usually post-viral vs a/w neuroblastoma. Tx is steroids/immunosuppression. |
| Hereditary hyperekplekia gene | Autosomal dominant GLRA1 glycine receptor. Recall glycine receptors = chlorine transport |
| Paroxysmal kinetic vs non-kinetic dyskinesia | Kinetic = post-stimulation, PRRT2 gene. Non-kinetic = random, MR1 gene |
| KCNA1 voltage-gated K-channel | Episodic Ataxia type 1 |
| CACNA1A Calcium channel | Episodic Ataxia type 2 |
| Episodic Ataxia type 1 vs 2 | Both ataxia. EA1 = ~2mins nystag/vertigo, K-channel. EA2 = hours/days vertigo, Ca-channel |
| Interictal myokymia | Myokymia = constant twitching (often eyelids). Seen in Episodic Ataxia |
| Episodic Ataxia treatment | EA1 = K-channel = acetazolamide. EA2 = Ca-channel = Dalfampiridine (K-blocker) |
| Isaac syndrome antibody | VGKC voltage-gated K-channel. Often paraneoplastic |
| Cancer + peripheral nerve hyperactivity | Isaac syndrome. VGKC antibody |
| Isaac syndrome EMG | Myokymic (short bursts, gun fire) and neuromyotonic (wax-wane, dive bomber) discharges |
| Isaac syndrome treatment | CBZ, phenytoin, IVIG/PLEX |
| Cancer + peripheral nerve hyperactivity + CNS findings | Morvan syndrome (aquired autoimmune neuromyotonia). ~Isaacs + memory/hallucinations/etc |
| Morvan syndrome antibody | Anti-CASPR2, Anti-LGi1 |
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