Familial periodic paralysis
- Article
- 2021-03-09
Familial periodic paralysis is a group of inherited neurological disorders caused by mutations in genes that regulate sodium and calcium channels in nerve cells. They are characterized by episodes in which the affected muscles are floppy, weak, and unable to contract. The affected muscles usually work normally between attacks.
The two most common types of periodic paralysis are:
Hypokalemic periodic paralysis is characterized by a drop in the level of potassium in the blood. In individuals with this mutation, seizures often begin in adolescence and are triggered by vigorous exercise, high carbohydrate meals, or by injection of insulin, glucose or epinephrine. Weakness can be mild and limited to certain muscle groups, or more severe and affect the arms and legs. Attacks can last for several hours or last for several days. Some patients can develop chronic muscle weakness later in life.
Hyperkalemic periodic paralysis is characterized by an increase in the level of potassium in the blood. Attacks often begin in childhood or early childhood and are accelerated by rest after exercise or fasting. Seizures are usually shorter, more frequent, and less severe than the hypokalemic form. Muscle spasms are common.
Therapy
Treatment of the periodic paralysis is aimed at preventing further attacks and relieving acute symptoms. Avoiding carbohydrate-rich meals and vigorous exercise and taking acetazolamide daily can prevent hypokalemic attacks. Seizures can be managed by drinking potassium chloride drink. Eating carbohydrate-rich, low-potassium foods and avoiding vigorous exercise and fasting can help prevent hyperkalemic attacks. Dichorfenamide can prevent seizures.
Prognosis
The prognosis for the familial periodic paralysis varies. Chronic attacks can result in progressive weakness that persists between attacks. Some cases respond well to treatment, which can prevent or reverse progressive muscle weakness.
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