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Kolkata siblings victim of rare brain disorder

Three brothers have been diagnosed with a rare degenerative brain disorder, which doctors say is normally found in one in 10 lakh people, reports Subhendu Maiti.
Hindustan Times | By Subhendu Maiti
UPDATED ON JUL 12, 2007 04:12 AM IST

Three brothers have been diagnosed with a rare degenerative brain disorder, which doctors say is normally found in one in 10 lakh people.

What is white matter disease?

Disorders that involve cerebral white matter, either exclusively or in combination with grey matter changes. One of them is pelizaeus merzbacher disease.

Pelizaeus merzbacher disease: A rare, progressive, degenerative central nervous system disorder in which coordination, motor abilities, and intellectual functions deteriorate. The growth of the fatty covering that wraps around and protects nerve fibres in the brain is affected.

Cause: A mutation in the gene that controls the production of a myelin protein called proteolipid protein (PLP).

Symptoms: Rapid, involuntary, rhythmic jerking of eyes and head, paralysis of the legs with hyperactive tendon reflexes, lack of coordination in the arms and legs, tremor, failure to develop normal control of head movement, deteriorating speech and mental function.

Treatment: No cure. No standard course of treatment.

Prognosis: Poor in severe cases. Progressive deterioration until death.

Doctors at a Kolkata hospital who examined Sailesh Chowhan, 12, Nirad, 4, and seven-month-old Joy said the three brothers suffered from ‘pelizaeus merzbacher,’ a genetic disorder that affects the brain. They have now decided to consult internationally acclaimed institutes abroad.

The three — from Jagaddal near Kolkata — were brought to the Institute of Child Health at Park Circus about 15 days ago with symptoms like rolling eyeball movement, drying up of the nervous system and severe walking disability.

After thorough investigations, doctors, led by institute director Dr Apurba Ghosh, were surprised to discover that all three suffered from the ailment. The institute then decided to send the diagnostic report to two top international institutes dealing with white matter disease in the US and Holland and seek their help.

Dr Ghosh told HT, “This is one of the rarest cases. We are still baffled how three brothers got affected with similar symptoms. We have already spoken to some experts on neuro-genetic science. Blood samples of the three children will also be sent to specialist laboratories in the US.”

Nirad and Joy have been discharged but Sailesh is still undergoing treatment, doctors at the hospital said.

Bidyadevi said all her children were afflicted within a month of their birth. The children were taken to many hospitals — BC Roy Polio Clinic, BC Roy Children’s Hospital, NRS Medical College, Bangur Institute of Neurology in Kolkata and All India Institute of Medical Sciences in Delhi — but doctors could not pinpoint the disorder. “Now I have come to this hospital. I have already spent more than Rs 40,000 on their treatment. I have sold all my ornaments. My husband has deserted us… my father who is a daily-wage labourer helps us,” she said.

“Here at the ICH, treatment is being done free of cost but I do not know how long I will live with my ailing sons,” she said. Dr Shyamal Das, an expert in neuro-medicine, when asked about the disease, said: “This is a very rare genetic disorder. We usually find only one among 10 lakh getting affected by the disease. We are more surprised as three brothers have contracted the disease.”

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