Tuesday, November 19, 2013

X-Link Dystonia Parkinsonism in Panay Island, Philippines

Medication induced Dystonia

Last week, My wife and I visited our neurologist who specialized in movement disorder, dystonia and Parkinson Disease. During our visit we were able to discuss a little bit of Lubag a neurological disorder endemic to Panay Island in the Philippines, where I grew up. I have seen one case of Dystonia during my teenage years in Iloilo, Panay Island. In case you have not heard of Dystonia, here's some information I found in the Web.

Dystonia is a neurological movement disorder, in which sustained muscle contractions cause twisting and repetitive movements or abnormal postures. The disorder may be hereditary or caused by other factors such as birth-related or other physical trauma, infection, poisoning (e.g., lead poisoning) or reaction to pharmaceutical drugs, particularly neuroleptics. Treatment is difficult and has been limited to minimizing the symptoms of the disorder, since there is no cure available. My curiosity on the X-linked dystonia lead me to the following article in the web as follows:

X-linked dystonia-parkinsonism (XDP) is a genetic form of dystonia found almost entirely among males of Filipino descent specifically in Panay Island. There are two terms used to describe this genetic disease endemic to Panay Island in the Philippines: XDP and Lubag. Lubag means twisted in the Ilonggo dialect of Panay. I know of a distant relative who had this disease when I was growing up in Panay Island in the 1960's. The disease is characterized by both dystonia and parkinsonism including signs and symptoms such as slow movement (bradykinesia), tremor, rigidity, and a loss of postural reflexes.

XDP occurs throughout the Philippines but is also diagnosed in the US and Canada in people of Filipino descent. All known cases of XDP originate from one common ancestor. The gene associated with XDP, called the DYT3 gene, was discovered only in 2003.

Treatment for XDP involves using medications to address dystonia, parkinsonism, or both. Parkinsonism symptoms may slightly improve with levodopa or dopamine agonist therapy, and dystonic features may respond to anticholinergics or benzodiazepines such as clonazepam (Klonopin). Zolpidem and tetrabenazine may be used if dystonia symptoms become multifocal or generalized. Botulinum toxin injections may improve focal dystonias in some cases.

For more details visit, http://www.dystonia-foundation.org/pages/more_info___x_linked_dystonia_parkinsonism

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