Westphall Disease

Below you will find more information about Westphall Disease from Medigest. If you believe that you are suffering from any of the symptoms of Westphall Disease it is important that you obtain an accurate diagnosis from a medical professional to ensure that you obtain the correct medication or treatment for your condition. There are medical conditions that carry similar symptoms associated with Westphall Disease and therefore the information provided by Medigest is offered as a guideline only and should never be used in preference to seeking professional medical advice. The information relating to Westphall Disease comes from a third party source and Medigest will not be held liable for any inaccuracies relating to the information shown.

Definition

Westphall disease otherwise known as the Hypokalemic periodic paralysis is a rare channelopathy where there is weakness in the muscle and even cause paralysis accompanying a fall in the level of potassium in the blood. It often begins in adolescence and is usually triggered of strenuous exercise followed by rest, high carbohydrate meals and sudden changes in the temperature.

Diagnosis

Diagnosis includes the use of Compound Muscle Amplitude Potential or the CMAP test and the exercise EMG or X-EMG. Also examination of patient's history or report of serum potassium low normal and genetic diagnosis.

Treatment

Treatment includes mainly on preventing further attacks and relieving acute symptoms. Administration of Acetazolamide or carbonic anhydrase inhibors along with a diet which avoids carbohydrate-rich meals and strenuous exercises are also advised.

Symptoms and Signs

Symptoms includes muscle weakness, muscle stiffness and muscle cramps.

Causes

It is caused by mutations in the dihydropyridine receptor calcium channel encode at chromosome 1q31-q32 (261, 262) (Miller,N.,Burton,F, et al. (2005). Walso and Hoyt's Clinical Neuro-Opthalmology, Lippincott Williams and Wilkins, p.1104). It is an inherited disorder.

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