L.B. NOVIKOVA, R.F. LATYPOVA, K.M. SHARAPOVA, A.P. AKOPIAN

 Bashkir State Medical University, Ufa

Contact details:

Sharapova K.M. – Ph. D. (Medicine), Associate Professor of the Department of Neurology and Neurorehabilitation

Address: 3 Lenin St., Ufa, Russian Federation, tel.: +7 (347) 273-61-92, e-mail: sharapovakarina.2020@gmail.com

The article presents a clinical case of a rare hereditary Hallervorden — Spatz disease with an atypical late form with onset in the fourth decade of life with a rapidly progressive course and unfavorable prognosis. The most characteristic signs of the disease in this patient were parkinsonism syndrome, pyramidal signs, various types of hyperkinesis, decreased cognitive functions, and depression. The diagnosis was confirmed by a clinical and a typical MRT signs in the form of the «tiger’s eye» symptom. There is no etiological and pathogenetic therapy for this disease; patients receive symptomatic treatment, the prognosis is unfavorable.

Key words: Hallervorden — Spatz disease, neurodegeneration, parkinsonism, «tiger’s eye» symptom, iron deposition.

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