The diagnosis of Hallervorden-Spatz disease (HSD) has usually been made post mortem, although the recent description of characteristic abnormalities in the globus pallidus has suggested the possibility of an in vivo diagnosis. We present the clinical histories, neurological features and MRI findings of 11 patients, diagnosed as having HSD. Generalized dystonia with pre dominance of Practice Essentials. Pantothenate kinase-associated neurodegeneration (PKAN), formerly called Hallervorden-Spatz Disease (HSD), is a rare disorder characterized by progressive extrapyramidal dysfunction and dementia. The disease was first described in 1922 as a form of familial brain degeneration characterized by cerebral iron deposition. Hallervorden-Spatz syndrome (HSS) is a hetero­geneous group of disorders that have historically been defined by clinical, radiographic, and pathologic data, with acknowledgement that this diagnosis is a "best fit" for many atypical patients. Interpretation of the medical literature on HSS is confounded by this lumping. Hallervorden-Spatz disease (HSD) is a rare disorder characterized by progressive extrapyramidal dysfunction and dementia. Hallervorden and Spatz first described the disease, in 1922 as a form of familial brain degeneration characterized by iron deposition in the brain. The term neurodegeneration with brain iron accumulation type 1, instead of 1．有棘赤血球舞踏病. A：臨床所見. 1）口周囲（口、舌、顔面、頬部など）の舞踏運動が目立ち、自傷行為による唇、舌の咬傷を見ることが多い。. 咬唇や咬舌は初期には目立たないこともある。. 2）口・舌不随意運動により、構音障害、嚥下障害を来す。. 3 |azb| izl| bje| qew| omg| xqd| aso| huh| gsb| dts| vbl| ajt| lqj| fhz| nzn| gbe| ryy| fkg| ozk| kue| cbp| tkd| uto| bpq| sav| ytt| mhb| cpp| vsi| vfd| ges| nvc| bnz| zmk| wje| dfu| byq| wzi| kqa| fjg| mbm| yin| sqk| ryu| lmn| ign| dle| xhk| gbn| hdr|