Cambios neuropsicológicos y neurofisiológicos en la enfermedad de Fahr: Report of three sisters

Background: Fahrs disease (basal ganglia calcification) is characterized by bi hemispherical calcium deposition in basal ganglia, dentate nucleus and semioval center. Its clinical manifestations are a rigid hypokinetic syndrome, mood disorders and cognitive impairment. Aim: To report to the results...

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Autores principales:	Cartier R,Luis, Passig V,Claudia, Gormaz W,Adriana, López C,Javier
Lenguaje:	Spanish / Castilian
Publicado:	Sociedad Médica de Santiago 2002
Materias:	Basal ganglia diseases Cognitive disorders Dementia
Acceso en línea:	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872002001200008
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spelling	oai:scielo:S0034-988720020012000082003-02-05Cambios neuropsicológicos y neurofisiológicos en la enfermedad de Fahr: Report of three sistersCartier R,LuisPassig V,ClaudiaGormaz W,AdrianaLópez C,Javier Basal ganglia diseases Cognitive disorders Dementia Background: Fahrs disease (basal ganglia calcification) is characterized by bi hemispherical calcium deposition in basal ganglia, dentate nucleus and semioval center. Its clinical manifestations are a rigid hypokinetic syndrome, mood disorders and cognitive impairment. Aim: To report to the results of a neurological assessment of three siblings with Fahr disease. Patients and methods: Three sisters, aged 55, 56 and 58 years, were studied. All had a rigid hypokinetic clinical picture associated with cerebellar involvement and a cognitive impairment that progressed in 8, 6 and 10 years respectively. Brain CAT scans showed symmetric and extensive calcifications of cerebellar white matter and dentate nuclei, pons, mesencephalon, lenticular nuclei, thalami and semioval centers. Hypoparathyroidism was ruled out. Cognition was assessed with WAIS and Benton tests and Weschler memory scale. The time of reaction to visual stimuli was studied. The processing speed of visual information and the interhemispheric conduction time of such information, were calculated. Cognitive evoked potentials (P 300) were also studied. Results: Cognitive impairment involved verbal and visual-spatial memory, planning, attention and concentration capacities and visual constructive skills. There was a prolongation of reaction time latencies and loss of the normal asymmetry of interhemispheric transmission (without right to left facilitation). P 300 evoked potentials were absent. Conclusions: These observations suggest that the pathogenesis of cognitive and motor changes in Fahrs disease is based in a dysfunction of cortico basal connections and their interhemispheric relations. This defines a subcortical dementia secondary to mineral deposits in subcortical structures ( Rev Méd Chile 2002; 130: 1383-90).info:eu-repo/semantics/openAccessSociedad Médica de SantiagoRevista médica de Chile v.130 n.12 20022002-12-01text/htmlhttp://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872002001200008es10.4067/S0034-98872002001200008
institution	Scielo Chile
collection	Scielo Chile
language	Spanish / Castilian
topic	Basal ganglia diseases Cognitive disorders Dementia
spellingShingle	Basal ganglia diseases Cognitive disorders Dementia Cartier R,Luis Passig V,Claudia Gormaz W,Adriana López C,Javier Cambios neuropsicológicos y neurofisiológicos en la enfermedad de Fahr: Report of three sisters
description	Background: Fahrs disease (basal ganglia calcification) is characterized by bi hemispherical calcium deposition in basal ganglia, dentate nucleus and semioval center. Its clinical manifestations are a rigid hypokinetic syndrome, mood disorders and cognitive impairment. Aim: To report to the results of a neurological assessment of three siblings with Fahr disease. Patients and methods: Three sisters, aged 55, 56 and 58 years, were studied. All had a rigid hypokinetic clinical picture associated with cerebellar involvement and a cognitive impairment that progressed in 8, 6 and 10 years respectively. Brain CAT scans showed symmetric and extensive calcifications of cerebellar white matter and dentate nuclei, pons, mesencephalon, lenticular nuclei, thalami and semioval centers. Hypoparathyroidism was ruled out. Cognition was assessed with WAIS and Benton tests and Weschler memory scale. The time of reaction to visual stimuli was studied. The processing speed of visual information and the interhemispheric conduction time of such information, were calculated. Cognitive evoked potentials (P 300) were also studied. Results: Cognitive impairment involved verbal and visual-spatial memory, planning, attention and concentration capacities and visual constructive skills. There was a prolongation of reaction time latencies and loss of the normal asymmetry of interhemispheric transmission (without right to left facilitation). P 300 evoked potentials were absent. Conclusions: These observations suggest that the pathogenesis of cognitive and motor changes in Fahrs disease is based in a dysfunction of cortico basal connections and their interhemispheric relations. This defines a subcortical dementia secondary to mineral deposits in subcortical structures ( Rev Méd Chile 2002; 130: 1383-90).
author	Cartier R,Luis Passig V,Claudia Gormaz W,Adriana López C,Javier
author_facet	Cartier R,Luis Passig V,Claudia Gormaz W,Adriana López C,Javier
author_sort	Cartier R,Luis
title	Cambios neuropsicológicos y neurofisiológicos en la enfermedad de Fahr: Report of three sisters
title_short	Cambios neuropsicológicos y neurofisiológicos en la enfermedad de Fahr: Report of three sisters
title_full	Cambios neuropsicológicos y neurofisiológicos en la enfermedad de Fahr: Report of three sisters
title_fullStr	Cambios neuropsicológicos y neurofisiológicos en la enfermedad de Fahr: Report of three sisters
title_full_unstemmed	Cambios neuropsicológicos y neurofisiológicos en la enfermedad de Fahr: Report of three sisters
title_sort	cambios neuropsicológicos y neurofisiológicos en la enfermedad de fahr: report of three sisters
publisher	Sociedad Médica de Santiago
publishDate	2002
url	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872002001200008
work_keys_str_mv	AT cartierrluis cambiosneuropsicologicosyneurofisiologicosenlaenfermedaddefahrreportofthreesisters AT passigvclaudia cambiosneuropsicologicosyneurofisiologicosenlaenfermedaddefahrreportofthreesisters AT gormazwadriana cambiosneuropsicologicosyneurofisiologicosenlaenfermedaddefahrreportofthreesisters AT lopezcjavier cambiosneuropsicologicosyneurofisiologicosenlaenfermedaddefahrreportofthreesisters
_version_	1718436070769557504

Cambios neuropsicológicos y neurofisiológicos en la enfermedad de Fahr: Report of three sisters

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