[Hallervorden-Spatz syndrome. Differential diagnosis of early onset dementia].
We report the history of a 38 year old patient who began to develop mental deterioration at the age of 26. After a time of 7 years neurological signs like writing dystonia occurred. Hallervorden-Spatz-Disease (HSD) was diagnosed at the age of 36 in vivo with the clinical presentation of severe dystonia, rigidity, dementia, and typical signal loss in the globus-pallidus the reticular part of the substantia nigra, and the nucleus ruber in the T-2 weighted MRI. The "eye-of-the-tiger"-sign, a bilateral hyperintensity in the rostral globus pallidus, was not observed in follow-up examinations. HSD is a rare autosomal-recessive or sporadic disease of unknown etiology. In one third of the patients a dementing process is the first clinical sign of the disorder, and is a rare differential diagnosis of early onset dementia.
Lechner
C
C
Abteilung für Psychiatrie, Krankenhaus Neukölln, Berlin.
Meisenzahl
E M
EM
Uhlemann
H
H
Helber-Böhlen
H
H
Fähndrich
E
E
D000328:Adult; D001284:Atrophy; D001921:Brain / Q000473:pathology*; D003072:Cognition Disorders / Q000209:etiology; D003704:Dementia / Q000175:diagnosis* / Q000209:etiology; D003937:Diagnosis, Differential; D018450:Disease Progression; D004421:Dystonia / Q000209:etiology*; D005917:Globus Pallidus / Q000473:pathology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D006211:Pantothenate Kinase-Associated Neurodegeneration / Q000150:complications / Q000175:diagnosis*; D012012:Red Nucleus / Q000473:pathology; D013378:Substantia Nigra / Q000473:pathology; D019690:Technetium Tc 99m Exametazime; D015899:Tomography, Emission-Computed, Single-Photon
D019690:Technetium Tc 99m Exametazime
Die Hallervorden-Spatz-Erkrankung. Eine Differentialdiagnose des präsenilen Demenzsyndroms.