کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
8462505 | 1549034 | 2009 | 9 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
Widespread thalamic and cerebellar degeneration in a patient with a complicated hereditary spastic paraplegia (HSP)
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کلمات کلیدی
موضوعات مرتبط
علوم زیستی و بیوفناوری
بیوشیمی، ژنتیک و زیست شناسی مولکولی
بیولوژی سلول
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چکیده انگلیسی
The hereditary spastic paraplegias (HSP) are a heterogeneous group of familial movement disorders sharing progressive spastic paraplegia as a common disease sign. In the present study, we performed the first pathoanatomical investigation of the central nervous degeneration of a female patient with a complicated HSP form who suffered from progressive spastic paraplegia, dysarthria, emotional symptoms, cognitive decline and a variety of additional neuropsychological deficits. This pathoanatomical investigation revealed in addition to loss of layer V Betz pyramidal cells in the primary motor cortex, widespread cerebellar neurodegeneration (i.e., loss of Purkinje cells and neuronal loss in the deep cerebellar nuclei), extensive and severe neuronal loss in a large number of thalamic nuclei, involvement of some brainstem nuclei, as well as damage to descending (i.e., lateral and ventral corticospinal tracts) and ascending (i.e., dorsal and ventral spinocerebellar tracts, gracile fascicle) fiber tracts. In view of their known functional role, damage to these central nervous gray and white matter components offers explanations for the patient's pyramidal signs, her cerebellar, psychiatric and neuropsychological disease symptoms.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Annals of Anatomy - Anatomischer Anzeiger - Volume 191, Issue 2, April 2009, Pages 203-211
Journal: Annals of Anatomy - Anatomischer Anzeiger - Volume 191, Issue 2, April 2009, Pages 203-211
نویسندگان
K. Seidel, Rai De Vos, L. Derksen, P. Bauer, O. Riess, W. den Dunnen, T. Deller, G. Hageman, U. Rüb,