[Corticobasal degeneration. The significance of clinical criteria for establishing the diagnosis].
Neuropathological studies show that about 20% of all patients suffering from an acinetic-rigid syndrome can not be given the diagnosis of idiopathic Parkinson's disease. Among these non-idiopathic Parkinson-syndromes the corticobasal degeneration (CBD) can be regarded as a separate disease entity. The pathological findings of moderate predominantly frontal and parietal cerebral atrophy, cortical Pick-cells and specific corticobasal inclusion bodies are considered valuable features which support the diagnosis. The clinical Characteristics of CBD are demonstrated in 3 patients including an acinetic-rigid syndrome, limb apraxia and "alien limb"-syndrome, as well as reflex myoclonus. Eye movement disorders, dementia and other rare symptoms may also be present. Electrophysiological reflex-testing helps to corroborate diagnosis. These findings and a summary which includes the previously published cases of CBD show that CBD in most cases can be diagnosed intra vitam.
Feifel
E
E
Neurologische Klinik und Poliklinik, Universität Freiburg.
Brenner
M
M
Teiwes
R
R
Lücking
C H
CH
Deuschl
G
G
D000368:Aged; D001284:Atrophy; D001479:Basal Ganglia / Q000473:pathology / Q000503:physiopathology*; D001480:Basal Ganglia Diseases / Q000175:diagnosis* / Q000473:pathology / Q000503:physiopathology; D002540:Cerebral Cortex / Q000473:pathology / Q000503:physiopathology*; D003937:Diagnosis, Differential; D004569:Electroencephalography; D004576:Electromyography; D005260:Female; D006801:Humans; D002479:Inclusion Bodies / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009127:Muscle Rigidity / Q000175:diagnosis / Q000473:pathology / Q000503:physiopathology; D009207:Myoclonus / Q000175:diagnosis / Q000473:pathology / Q000503:physiopathology; D009410:Nerve Degeneration / Q000502:physiology*; D009460:Neurologic Examination; D010302:Parkinson Disease, Secondary / Q000175:diagnosis* / Q000473:pathology / Q000503:physiopathology; D012021:Reflex, Abnormal / Q000502:physiology; D013494:Supranuclear Palsy, Progressive / Q000175:diagnosis / Q000473:pathology / Q000503:physiopathology
Kortiko-basale Degeneration. Die Bedeutung klinischer Kriterien zur Diagnosestellung.