Fahr Disease, a rare disorder, may be fatal if early detection is missed, say experts 

By Faruque Ahamed 

KOLKATA, 19 FEBRUARY – 

Fahr Disease (FD), a rare inherited or sporadic neurological disorder with a prevalence of hardly one case in every ten lakh population, requires more research works for the treatment of patients so that they can have an extended life span. 

Basal ganglia calcification in brain is also known as FD that invented first by German neurologist Karl Theodor Fahr in 1993. It is characterised by abnormal deposition of calcium in areas of the brain that control movements including basal ganglia, thalamus, dentate nucleas, and cerebral cortex. 

FD may also present with cerebellar dysfunction, speech difficulty, dementia and neuro-psychiatric symptoms among patients. 

Basal ganglia that commonly affects PARKINSON disease also plays many roles in cognition, including facial expression (his is the region of brain that helps mynah birds talk). This type of patient may also show the symptoms of disorder, meaning alternate depression and excitement very common mental health condition, whose cause remains unknown.

US- based NRI Dr Yamini Atluri’s work done in Michigan provides insight into the region of the brain affected in bipolar disorder, which was until now not known properly.

“The incidence of basal ganglia calcifications on CT is about 0.93 % but the true incidence of Fahr Disease itself is unknown and too low to even be quoted,” Dr Atluri associated with Spectrum Health in Michigan said. 

She conducted research work on a 56 year-old female patient with a history of bipolar disorder presented with symptoms of acute altered mental status. A CT scan on brain revealed that the patient had bilateral dense calcifications in the basal ganglia and dentate nuclei characteristic for Fahr syndrome. 

“FD is an incidental radiological findings and patients may be asymptomatic. Common symptoms of the disease include Parkinson-like disorders in movement, mood and personality, progressive dementia, anterograde amnesia, headache, vertigo, speech abnormality and seizures,” according to her. 

Her research also revealed that bipolar disorder could be an early manifestation of FD that is usually missed at the initial diagnosis.

“FD is a very rare incurable neurological syndrome that requires more research works for proper treatment so that affected patients can have longer life. In my more than 40 years’ professional career as a neurologist I have treated hardly three to four Fahr disease patients including an Army personnel,” said Dr Trishit Roy, former director of the government Bangur Institute of Neurology (BIN) in Kolkata. 

“With the use of modern hi-tech medical devises like CT scan the detection of the disease is being reported nowadays but the treatment facilities are not up to the mark. Proper detection of the disease should be carefully taken into account,” Dr Roy added.