S K Mathur and colleagues have done some excellent case report tracking a very rare syndrome Fahr's Syndrome is a rare, genetically dominant, inherited neurological disorder characterized by abnormal deposits of calcium in areas of the brain that control movement, including the basal ganglia and the cerebral cortex. First described in 1930 by German neurologist Karl Fahr .The basal ganglia calcification may occur as a consequence of several known genetic conditions affecting locus at 14q, chromosome 8 and chromosome 2. It usually manifests itself in the third to fifth decade of life but may appear in childhood or later in life. It is usually identified on CT scan but may be visible on plain films of the skull. Making a clinical diagnosis of Fahr's disease relies on the combination of clinical features, brain imaging, and exclusion of other causes of intracranial calcification. It usually presents with Neuropsychiatric symptoms manifestations includes clumsiness, fatigability, unsteady gait, slow or slurred speech, dysphagia, involuntary movements or muscle cramping. Seizures of various types are common. Fahr's disease is a differential diagnosis of frontal lobe syndrome. The available treatment is directed symptomatic control. Currently no standard course of treatment available. Genetic counseling may be helpful.
References 1. http://rarediseases.info.nih.gov/GARD/Disease.aspx?PageID=4&diseaseID=8272 2. JNNP - Sign In Page 3. T. Fahr: Idiopathische Verkalkung der Hirngef?sse. Zentralblatt f?r allgemeine Pathologie und pathologische Anatomie, 1930-1931, 50: 129-133. 4. http://www.whonamedit.com/synd.cfm/451. 5.Dr Osama Hammer MBBch.,MSc.,MRCPsych
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