No abstract available.
Hypokalemic Periodic Paralysis*

No abstract available.
Electrodiagnosis ; Hypokalemic Periodic Paralysis ; Neural Conduction* ; Paralysis* ; Thyrotoxicosis

Recognizing the underlying causes of hypokalemic paralysis seems to be essential for the appropriate management of affected patients and their prevention of recurrent attacks. There is, however, a paucity of documented reports on the etiology of hypokalemic paralysis in Korea. We retrospectively analyzed 34 patients with acute flaccid weakness due to hypokalaemia who were admitted during the 5-year study period in order to determine the spectrum of hypokalemic paralysis in Korea and to identify the differences in clinical parameters all across the causes of hypokalemic paralysis. We divided those 34 patients into 3 groups; the 1st group, idiopathic hypokalemic periodic paralysis (HPP), the 2nd, thyrotoxic periodic paralysis (TPP), and the 3rd group, secondary hypokalemic paralysis (HP) without TPP. Seven of the patients (20.6%) were diagnosed as idiopathic HPP considered the sporadic form, and 27 patients (79.4%) as secondary HP. Among the patients diagnosed as secondary HP, 16 patients (47.1%) had TPP. Patients of secondary hypokalemic paralysis without TPP required a longer recovery time compared with those who had either idiopathic HPP or TPP. This is due to the fact that patients of secondary HP had a significantly negative total body potassium balance, whereas idiopathic HPP and TPP were only associated with intracellular shift of potassium. Most of the TPP patients included in our study had overt thyrotoxicosis while 3 patients had subclinical thyrotoxicosis. This study shows that TPP is the most common cause of hypokalemic paralysis in Korea. And we suggest that doctors should consider the presence of TPP in patients of hypokalemic paralysis even if they clinically appear to be euthyroid state.
Humans ; Hypokalemic Periodic Paralysis ; Korea ; Paralysis ; Potassium ; Retrospective Studies ; Thyrotoxicosis

Two patients of a family, suffenng from frequent periodic paralysis, were evaluated. Their family history reveals seven affected members in three generations, with autosomal dominance pattern. Their earliest symptoms were noticed between 10-13 years of age. The serum potassium level fell during the attack. EMG demonstrated progresslve reduction in numer of motor units and drop off in the response of muscle fibers to stimuli. These findings were consistent with familial hypokalemic periodic paralysis. The clinical features, electron microscoscopic findings, and pathogenesis were also described.
Family Characteristics ; Humans ; Hypokalemic Periodic Paralysis* ; Paralysis ; Potassium

No abstract available.
Acetazolamide ; Acidosis ; Acidosis, Renal Tubular ; Hypokalemic Periodic Paralysis ; Muscle Weakness

Hyperthyroidism has been associated with changes in muscle function such as thyrotoxic myopathy, thyrotoxic periodic paralysis and thyroid opthalomopathy, but rarely rhabdomyolysis. Usually serum creatinine kinase is either normal or low in hyperthyroidism. Only 3 reports described association between rhabdomyolysis and hyperthyroidism, no previous literatures have thyrotoxic periodic paralysis associated rhabdomyolysis. Patients with hypokalemic periodic paralysis sometimes elevated serum muscle protein during recovery from paralytic attack, but the mechanism was not well known. We report a patient who presented with clinical feature of thyrotoxic periodic paralysis and increasing serum CK, myoglobin during recovery from paralysis.
Creatinine ; Humans ; Hyperthyroidism ; Hypokalemic Periodic Paralysis ; Muscle Proteins ; Muscular Diseases ; Myoglobin ; Paralysis* ; Phosphotransferases ; Rhabdomyolysis* ; Thyroid Gland

Primary hypokalemic periodic paralysis (HOKPP) is an autosomal dominant disorder manifesting as recurrent periodic flaccid paralysis and concomitant hypokalemia. HOKPP is divided into type 1 and type 2 based on the causative gene. Although 2 different ion channels have been identified as the molecular genetic cause of HOKPP, the clinical manifestations between the 2 groups are similar. We report the cases of 2 patients with HOKPP who both presented with typical clinical manifestations, but with mutations in 2 different genes (CACNA1Sp.Arg528His and SCN4A p.Arg672His). Despite the similar clinical manifestations, there were differences in the response to acetazolamide treatment between certain genotypes of SCN4A mutations and CACNA1S mutations. We identified p.Arg672His in the SCN4A gene of patient 2 immediately after the first attack through a molecular genetic testing strategy. Molecular genetic diagnosis is important for genetic counseling and selecting preventive treatment.
Acetazolamide ; Genetic Counseling ; Genotype ; Humans ; Hypokalemia ; Hypokalemic Periodic Paralysis ; Ion Channels ; Molecular Biology ; Paralysis

Primary hypokalemic periodic paralysis (HOKPP) is an autosomal dominant disorder manifesting as recurrent periodic flaccid paralysis and concomitant hypokalemia. HOKPP is divided into type 1 and type 2 based on the causative gene. Although 2 different ion channels have been identified as the molecular genetic cause of HOKPP, the clinical manifestations between the 2 groups are similar. We report the cases of 2 patients with HOKPP who both presented with typical clinical manifestations, but with mutations in 2 different genes (CACNA1Sp.Arg528His and SCN4A p.Arg672His). Despite the similar clinical manifestations, there were differences in the response to acetazolamide treatment between certain genotypes of SCN4A mutations and CACNA1S mutations. We identified p.Arg672His in the SCN4A gene of patient 2 immediately after the first attack through a molecular genetic testing strategy. Molecular genetic diagnosis is important for genetic counseling and selecting preventive treatment.
Acetazolamide ; Genetic Counseling ; Genotype ; Humans ; Hypokalemia ; Hypokalemic Periodic Paralysis ; Ion Channels ; Molecular Biology ; Paralysis

We describe a 46-year-old woman with hypokalemic paralysis as the initial manifestation of Sjorgen's syndrome. Sjorgen's syndrome is an autoimmune exocrinopathy, characterized by keratoconjuntivitis sicca and xerostomia. Among the extraglandular manifestations of Sjorgen's syndrome, renal tubular involvement, especially renal tubular acidosis, is the most often latent or minimally symptomatic. Renal tubular acidosis is estimated to be present in 25~30 percent of the cases. Hypokalemic paralysis may serve as a clinical marker for more severe renal disease in patient who has primary Sjorgen's syndrome with renal tubular acidosis, even though it is a rare manifestation of Sjorgen's syndrome.
Acidosis, Renal Tubular ; Biomarkers ; Female ; Humans ; Hypokalemic Periodic Paralysis* ; Middle Aged ; Paralysis ; Xerostomia

Periodic paralysis associated with thyrotoxicosis is characterized by intermittent flaccid paralysis of the skeletal muscle. The paralysis usually involve the skeletal muscle of the limbs, especially lower extrimities. In general, sensory function is intact. Involvement of respiratory, ocular or bulbar muscles is very rare, but bulbar and respiratoy invelvement may prove fatal. It is very rare a case that has severe clinical manifestation such as cardiac arrest. We report a case of thyrotoxic hypokalemic periodic paralysis presenting as cardiac arrest.
Extremities ; Heart Arrest ; Hypokalemic Periodic Paralysis ; Muscle, Skeletal ; Muscles ; Paralysis ; Sensation ; Thyrotoxicosis