1 Wisniewski KE, French JH, Rosen JF, Koslowski PB, Tenner M, Wisniewski HM. Basal ganglia calcification (BGC) in Down's syndrome. Another manifestation of premature aging. Ann NYAcad Sci 1982;396:179-89. 2 Jakab I. Basal ganglia calcification and psychosis in mongolism. Eur Neurol 1978;17:300-14. 3 Lowenthal A, De Deyn P. Striopallidodentate calcifications. In: Joseph AB, Young RR, eds. Moveme...

Fahr's disease refers to a rare syndrome characterised by symmetrical and bilateral intracranial calcification. The basal ganglia are the most common site of involvement and most cases present with extra-pyramidal symptoms. We describe two men with Fahr's diseases who presented with prominent frontal lobe symptoms. The first man presented with frequent uncontrollable bursts of laughter and cryi...

BACKGROUND Primary familial brain calcification (PFBC) is a rare disorder characterized by distinctive bilateral brain calcification and variable clinical presentations. However, cerebrovascular attack was rarely reported in PFBC patients. We here reported a SLC20A2 mutation patient presenting with acute ischemic stroke. CASE PRESENTATION A 56 years old man was transferred to our hospital bec...

Two related infants with microcephaly, spastic quadriplegia, and profound retardation are reported. Both showed extensive bilateral symmetrical calcification of the basal ganglia with cerebrospinal fluid pleocytosis.

Dense symmetrical calcification of the corpus striatum is rare, but of some general interest for two reasons. First, in a significant number of the reported cases this apparently degenerative condition is closely linked with a metabolic disorder, hypoparathyroidism, which may be idiopathic, parathyroprivic, or of the variety in which the parathyroid glands are histologically normal but the body...

OBJECTIVE To determine the neuroradiological abnormalities associated with subjects carrying the mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes (MELAS) tRNA(Leu)(UUR) A3243G point mutation METHODS Mitochondrial genetic analysis was performed on 24 subjects from six kindreds with the MELAS tRNA(Leu)(UUR) A3243G point mutation. Cerebral CT and MRI were performe...