Above suspicion 1995 paralyzed predicted
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Factors that trigger paralytic attacks (e.g., unusually strenuous effort, carbohydrate-rich meals or sweets, cold, stress/excitement/fear, high salt intake, prolonged immobility, oral or intravenous glucosteroids, certain anesthetic procedures, alcohol) should be avoided when possible.Įvaluation of relatives at risk. Renal ultrasound should be performed annually.Īgents/circumstances to avoid. For those taking acetazolamide, the following are indicated every three months: complete blood count electrolytes and glucose, uric acid, and liver enzyme levels. Periodic neurologic examination with attention to muscle strength in the legs should be performed to detect long-lasting weakness associated with myopathy. The frequency of consultations is adapted to the individual's signs/symptoms and response to preventive treatment. Individuals with known pathogenic variants in one of the genes associated with hypoPP who developed myopathy without having experienced episodes of weakness have been reported. It is unknown whether prevention of paralytic attacks also prevents the development of myopathy. Anesthetic complications should be prevented by strict control of serum potassium concentration, avoidance of large glucose and salt load, maintenance of body temperature and acid-base balance, and careful use of neuromuscular blocking agents with continuous monitoring of neuromuscular function. Creating a safe environment, getting help in case of paralytic attack, and preventing falls and accidents are critical an affected person experiencing a paralytic attack must have access to potassium as well as physical assistance and companions must be informed of the risk in order to enable rapid treatment. If carbonic anhydrase inhibitors are not tolerated or not effective after prolonged use, alternatives include potassium-sparing diuretics such as triamterene, spironolactone, or eplerenone. If dietary intervention and oral potassium supplementation are not effective in preventing attacks, treatment with a carbonic anhydrase inhibitor (acetazolamide or dichlorphenamide) may be necessary. This may be achieved by avoidance of triggering factors, adherence to a diet low in sodium and carbohydrate and rich in potassium, and with the use of oral potassium supplementation. The goal of preventive treatment is to reduce the frequency and intensity of paralytic attacks. There is no known curative treatment for hypoPP-related myopathy physiotherapy may help to maintain strength and motor skills.
#Above suspicion 1995 paralyzed predicted serial
Severe attacks typically require more intensive medical management with intravenous potassium infusion, serial measurement of serum potassium concentration, clinical evaluation of possible respiratory involvement, and continuous electrocardiogram monitoring. Moderate attacks may be self-treated in a non-medical setting by ingestion of oral potassium salts. Treatment varies depending on the intensity and duration of the paralytic attack.
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A myopathy may occur independent of paralytic symptoms and may be the sole manifestation of hypoPP. Long-lasting interictal muscle weakness may occur in some affected individuals and in some stages of the disease and in myopathic muscle changes. The age of onset of the first attack ranges from two to 30 years the duration of paralytic episodes ranges from one to 72 hours with an average of nearly 24 hours. Additional triggers can include cold, stress/excitement/fear, salt intake, prolonged immobility, use of glucosteroids or alcohol, and anesthetic procedures. The major triggering factors are cessation of effort following strenuous exercise and carbohydrate-rich evening meals. Some individuals have only one episode in a lifetime more commonly, crises occur repeatedly: daily, weekly, monthly, or less often. The episodes develop over minutes to hours and last several minutes to several days with spontaneous recovery. The paralytic attacks are characterized by decreased muscle tone (flaccidity) more marked proximally than distally with normal to decreased deep tendon reflexes. Hypokalemic periodic paralysis (hypoPP) is a condition in which affected individuals may experience paralytic episodes with concomitant hypokalemia (serum potassium <3.5 mmol/L).