Basal ganglia calcification (striopallidodentate calcification) and cognitive impairment

THESES

BASAL GANGLIA CALCIFICATION (STRIOPALLIDODENTATE CALCIFICATION) AND COGNITIVE IMPAIRMENT (ABSTRACT)^*. THESIS. NITEROI, 1998.

GILBERTO LEVY^**

Basal ganglia calcification (BGC), or striopallidodentate calcification, is a bilateral and symmetrical intracranial calcification, affecting, in decreasing frequency, the pallidum, dentate nucleus of cerebellum, striatum, thalamus, white matter and cerebral cortex. It is a syndromic condition, being associated with endocrine ¾ particularly hypoparathyroidism and pseudohypoparathyroidism ¾, toxic/iatrogenic and mitochondrial disorders; there are also instances of familiar and idiopathic BGC. The clinical manifestations include neuropsychiatric ¾ cognitive impairment and dementia, schizophreniform psychosis, and affective disorder ¾, epileptic, extrapyramidal and cerebellar manifestations. As regards the diagnostic workup, neuroimage, mainly cranial computerized tomography (CT), and laboratory exams, in order to investigate calcium-phosphorus metabolism abnormalities, are most important.

We have evaluated six patients with BGC on cranial CT (three with extensive BGC and three with pallidal calcification), through clinical, laboratory and neurological examinations, including extensive neuropsychological battery. Neuropsychological tests consisted of: mini-mental state examination (MMSE), Wechsler adult intelligence scale-revised (WAIS-R), Wechsler memory scale-revised (WMS-R), Wisconsin card sorting test (WCST), trail making test (TMT) A and B, Porteus tests, and evaluation of language, praxis and gnosia.

Results evidenced marked cognitive impairment in patients with extensive BGC, two of them displaying dementia and the third mental retardation, characterized by: impaired verbal, performance and total intelligence quocient (IQ); impairment of attention/concentration and memory; impairment of mental shift, abstract/conceptual reasoning and planning; preservation of language, except for dysnomia in one patient; preservation of ideomotor and ideational praxis, and gnosia; impaired constructional praxis. MMSE was not an adequate screening instrument for the detection of dementia in BGC patients. Patients with pallidal calcification presented impairment of attention/concentration, mental shift and abstract/conceptual reasoning, suggesting frontal dysfunction.

The concepts of subcortical dementia and physiological BGC are discussed. We recommend the utilization of the terminology "striopallidodentate calcification", "striopallidal calcification", "pallidodentate calcification" and "pallidal calcification".

KEY WORDS: basal ganglia calcification, striopallidodentate calcification, Fahr syndrome, cognition, subcortical dementia.

*

Calcificação dos núcleos da base (calcificação estriopalidodenteada) e comprometimento cognitivo (Resumo). Tese de Mestrado, Universidade Federal Fluminense (Área: Neurologia). Orientador: Osvaldo José Moreira do Nascimento.

**

Address: Avenida Delfim Moreira 90 / 201, 22441-000 Rio de Janeiro RJ, Brasil.

Publication Dates

• Publication in this collection
  06 Nov 2000
• Date of issue
  Mar 1999