PARKINSONS SYNDROME 70% Parkinson's d. 30% Other disease RED FLAGS - - MEDICATION (NEUROLEPTIC!!) ■ - positive familial history ■ - early falls, postural instability ■ - rapid progression ■ - nondoparesponsibility ■ - early autonomic disturbance ■ - oculomotor disturbance ■ - serious pseudobulbar syndrome ■ - early dementia ■ - separate leg disability ■ - pyramidal or cerebellar syndrome DIFFERENTIAL DIAGNOSIS IPN ■ A/ other NEURODEGENERATIV Disorders ■ ( PARKINSON PLUS d.) ■ - rare,nondoparesponsibility, rapid progression, serious prognossis 1. Synukleinopathy (MSA, DLBD)- intracytoplasmatic inclusion alfa-synuklein ■ 2. Tauopathy (PSP, CBD) - intracytoplasmatic inclusion tau-protein - B/ Other NEUROLOGIC Disorders ■ HD Westphal variant - familial ■ (Wilson's disease) ■ C/ SECUNDARY parkinson syndrome ■ Side effects of drug, vascular, NTH DRUG INDUCED Farmakologic anamnesis ■ TYPICAL NEUROLEPTIC !! ■ Haloperidol, Chlorpromazin,Chlorprotixen, Tisercin ■ TREATMENT: Akineton ■ ANTIEMETIC,PROKINETIC ■ Cerucal, Degan, Torecan ■ ANTIHISTAMINE ■ Prothazin BLOCK of Calcium channels cinarizin, flunarizin VASCULAR - OFTEN ■ MRI finding: multiinfarct changes -status lacunaris ■ - development gradually or as a stroke ■ - Pa sy UNILATERAL during the first year after stroke after stadium of hemiparesis ■ - Pa sy BILATERAL (frontal only gait disturbance ■ LOWER BODY parkinsonismus ■ other signs: pseudobulbar palsy, dementia,incontinency ■ - rapid progression - - TREATMENT : low effect of amantadin ■ NO! L-Dopa because of postsynaptic DA receptors lesion NORMOPRESSURE HYDROCEPHALUS ■ Disproportion : production X absorption of CSF (liquor pressure is not increased ) Old aged mostly men ■ Hakimo clinical features idementia urinal incontinency gait apraxia (bradykinezia,wide base) ■ MRI comunicated hydrocephalus - TREATMENT- lumbal punction (- 50ml CSF).... ■ gait improvement.....V-P shunt MSA Multiple system atrophy o STN.....first s. parkinson sy msa type P o opca....first s. cerebellar sy MSA type C o Shy-Drager sy....autonomic sy o (ortostatic hypotension, incontinency) MSA type A The possibility : combination or isolated o Initial stadium: 50% autonomic sy O 45% parkinson sy O ( 5% cerebellar sy) o SYNUKLEINOPATHY O - prevalence 5-15/100 000, 8% - cerebellar + brainstem SN,striatum,palidum, - ONUF's ncl (segment S2-S4) o - doparesponsibility in the early stadium 29%...differentiation from PD difficult O Clinical Feautures: dystonia, pseudobulbar palsy, inspiration stridor, polyneuropathy o - no dementia! m EXAMINATION o Brain MRI — cerebellar + brainstem atrophy o - EMG anal external sphincter - denervation cm Onuf s ncl. o - EMG for diagnostic Polyneuropathy o - test of Vegetative system (EKG variability R-R interval) O - Orthostatic test (BP while lying down and 1 min after posture .....differences 30/15) PSP PROGRESSIVE SUPRANUCLEAR PALSY o - prevalence 7/100 000, 7-12% o - midle and old age o - Atrophy mesencephalon + pons tegmentum + F polar part of cortex o TAUOPATHY CLINICAL FEATURES o - pa sy with dominant axial rigidity without tremor o - extended trunk o - early falls due to postural instability o - vertical gaze palsy, hypometric saccades o - apraxia eye lids, retraction of upper eye lids (surprised look) o - subcortical dementia (F lobe) o - pseudobulbar palsy o - dystonia (focal cervical) o - inspiratory stridor o - nondoparesponsibility EXAMINATION o - Hearing EP.....central lesion o - Brain MRI... mesencephalon atrophy o enlarged III. ventricule o DLBD Diffuse lewy body disease CBD CORTICOBASAL DEGENERATION o - prevalence 0,5% o - age over 70 years o - unilateral cortical atrophy F+P o - atrophy SN o o TAUOPATHY CLINICAL FEATURES o - contralateral hemiparkinsonism o - cortical (limb apraxia..alien hand syndrome, hemihypestesia, symbolic function disturbance) o - dementia o - cortical myoklonus o - dystonia o - pyramidal syndrome EXAMINATION o Brain MRI - asymetric cortical atrophy P + F o Brain PET - (hypometabolism F-P cortex) WESTPHAL variant Huntington D. o - Autosomal Dominant o - 5% patient of HD o - young age (manifestation before 20 years) o - the first symptom parkinsonism (rigidity + hypokinezia) o - later chorea + dementia o - rapid progression o - genetic test ADVANTAGE of DAT SCAN inpakkinson syndrome o PD asymetrie re-uptake in striatum o o MSA,PSP symetrie re-uptake in striatum + postsynaptic disturbance DLBD asymetrie re-uptake in striatum VASCULAR pa sy normal DRUG INDUCED pa sy normal