Stereotaktische Behandlung der Wilsonschen Krankheit

zweidimensionales bewegtes Bild

Vorstellung einer 31jährigen Patientin mit Wilsonscher Krankheit. Schwere rechtsseitige Intentionsataxie. Nach langjähriger medikamentöser Therapie keine Besserung des Krankheitsbildes. Erklärung des stereotaktischen Eingriffs (Trick). Beteiligung des Nucleus dentatus, Nucleus ruber, Pallidum und des motorischen Thalamusanteils. Beseitigung der ungeordneten Überaktivität ohne Beeinflussung der Willkürmotorik durch orobasale Thalamotomie. Vorstellung der Patientin sechs bzw. acht Jahre nach der Thalamotomie: Die extrapyramidal-motorischen Störungen sind fast vollständig verschwunden.
Stereotactic management is indicated in cases of the "pseudosclerotic" type of Wilson's disease when "positive" movement disorders of extra-pyramidal origin (after Purdon Martin), such as rigidity and hyperkinesia, fail to respond adequately to medical management because of irreversible damage to brain. Motor function can be permanently improved by placing precise lesions in the antagonistic systems activated or released by the disease process if sufficient postoperative treatment with Penicillamine and potassium sulfide prevents further storage of copper in the body and additional damage to the central nervous system. The film presents a female patient who, at the age of 26 years, developed a severe right-sided intention tremor. At 29 minor disturbances appeared on the left. She was totally incapacitated by this movement disorder, and medically-induced excretion of large amounts of copper yielded only slight improvement. A left thalamotomy performed at 32 years of age resulted in almost total disappearance of her right-sided symptoms, and the use of her right arm is virtually normal 8 years postoperatively. A trick film technique is used to illustrate both the interplay of the extra-pyramidal motor system and the site of malfunction in this patient. It also axplains the stereotaxic procedure that eliminated the motor dysfunction.

Object Details

• Alternative title: Stereotactic Treatment of Wilson's Disease
• Location: Hannover TIB
• Language: Deutsch
• Notes: Audiovisuelles Material

References and Relationships

• Bibliographic citation: Stereotaktische Behandlung der Wilsonschen Krankheit ; (Jan. 1974)

Classification and Topics

• Keyword: stereotaktische Behandlung ; Medizin ; Stereotactic treatment ; Hepatolenticular degeneration ; neurology and neurosurgery ; Wilson-Pseudosklerose ; Wilson's disease ; hepatolentikuläre Degeneration ; medicine ; Degenerative Hirnerkrankungen ; degenerative brain diseases (cerebrosis) ; Neurologie und Neurochirurgie

Contributors, Places and Time

• Event: Veröffentlichung
• (who): IWF (Göttingen)
• (when): 1974-01-01
• Contributor: Orthner, Hans ; Roeder, Fritz

Further information

• DOI: 10.3203/IWF/C-1129
• Last update: 04.12.2024, 8:09 AM CET