[Steele-Richardson-Olszewski syndrome].
BACKGROUND
Basal ganglia diseases with an akinetic-rigid symptom manifest like a Parkinson disease or an atypical Parkinson syndrome. Their symptoms are similar and therefore a diagnosis often is only possible after long-standing care of the patient. In the class of the atypical Parkinson syndromes is the progressive supranuclear palsy (PSP), which is also known as Steele-Richardson-Olszewski syndrome.


METHODS
A seventy-year-old patient who reported unaccountable sudden drops and disturbances of balance as well as diplopia in near vision was examined and a retrospective analysis of the case history was carried out.


RESULTS
Only long-standing observation of the patient rendered the suspected diagnosis of a progressive supranuclear palsy (PSP) possible. Only after determination of the clinic picture of a vertical palsy to the up and the down direction and a bad optokinetic nystagmus with an existing vertical vestibular reflex could the clinical diagnosis of PSP be confirmed.


CONCLUSIONS
In cases with the combination of a vertical palsy, an akinetic-rigid Parkinson syndrome, pyramidal tract signs and subcortical dementia, the diagnosis of a suspected progressive supranuclear palsy should be made. L-Dopa medication has very little or no influence on PSP. At present, however, a certain diagnosis is only possible post-mortem, in spite of helpful clinical diagnostics.
Pletz
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C
Institut für Orthoptik, Pleoptik und Neuroophthalmologie des Konventkrankenhauses der Barmherzigen Brüder, Linz (Primaria Dr. Barbara Neudorfer). claudia.pletz@bblinz.at
D000368:Aged; D001284:Atrophy; D001921:Brain / Q000473:pathology; D003937:Diagnosis, Differential; D004172:Diplopia / Q000209:etiology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009460:Neurologic Examination; D017120:Nystagmus, Optokinetic / Q000502:physiology; D015840:Oculomotor Nerve Diseases / Q000209:etiology; D012027:Reflex, Vestibulo-Ocular / Q000502:physiology; D012189:Retrospective Studies; D013494:Supranuclear Palsy, Progressive / Q000175:diagnosis*; D014787:Vision Tests