Abduction range: A potential parameter for the long exercise test in hypokalemic periodic paralysis during inter‐attack periods

2019 ◽  
Vol 61 (1) ◽  
pp. 104-107
Author(s):  
Lei Zhang ◽  
Jingwen Niu ◽  
Yi Li ◽  
Yuzhou Guan ◽  
Liying Cui ◽  
...  
Keyword(s):  
Exercise Test ◽  
Periodic Paralysis ◽  
Potential Parameter ◽  
Hypokalemic Periodic Paralysis

Morphologic alteration in the muscles of patients with hypokalemic periodic paralysis or myopathy

1990 ◽  
Vol 48 (3) ◽  
pp. 222-223
Author(s):  
T. Shimizu ◽  
Y. Muranaka ◽  
I. Ohta ◽  
N. Honda
Keyword(s):  
Clinical Manifestations ◽  
Quadriceps Muscle ◽  
Honeycomb Structure ◽  
Periodic Paralysis ◽  
Ultrastructural Changes ◽  
Hypokalemic Periodic Paralysis ◽  
Electron Microscopic ◽  
Tubular Aggregates ◽  
Hypokalemic Myopathy ◽  
Transmission Electron

There have been many reports on ultrastructural alterations in muscles of hypokalemic periodic paralysis (hpp) and hypokalemic myopathy(hm). It is stressed in those reports that tubular structures such as tubular aggregates are usually to be found in hpp as a characteristic feature, but not in hm. We analyzed the histological differences between hpp and hm, comparing their clinical manifestations and morphologic changes in muscles. Materials analyzed were biopsied muscles from 18 patients which showed muscular symptoms due to hypokalemia. The muscle specimens were obtained by means of biopsy from quadriceps muscle and fixed with 2% glutaraldehyde (pH 7.4) and analyzed by ordinary method and modified Golgimethod. The ultrathin section were examined in JEOL 200CX transmission electron microscopy.Electron microscopic examinations disclosed dilated t-system and terminal cistern of sarcoplasmic reticulum (SR)(Fig 1), and an unique structure like “sixad” was occasionally observed in some specimens (Fig 2). Tubular aggregates (Fig 3) and honeycomb structure (Fig 4) were also common characteristic structures in all cases. These ultrastructural changes were common in both the hypokalemic periodic paralysis and the hypokalemic myopathy, regardless of the time of biopsy or the duration of hypokalemia suffered.

ANAESTHESIA MANAGEMENT FOR A PATIENT WITH FAMILIAL HYPOKALEMIC PERIODIC PARALYSİS

2014 ◽  
Author(s):  
Özgül Keskin ◽  
Hatice Türe ◽  
Özge Köner ◽  
Ferdi Menda ◽  
Bora Aykaç
Keyword(s):  
Periodic Paralysis ◽  
Hypokalemic Periodic Paralysis

Hypokalemic periodic paralysis mutations: Confirmation of mutation and analysis of founder effect

1996 ◽  
Vol 6 (1) ◽  
pp. 27-31 ◽  
Author(s):  
Christie L.S. Grosson ◽  
Jesus Esteban ◽  
Diane McKenna-Yasek ◽  
James F. Gusella ◽  
Robert H. Brown
Keyword(s):  
Founder Effect ◽  
Periodic Paralysis ◽  
Hypokalemic Periodic Paralysis

scholarly journals Identification and Functional Characterization of Kir2.6 Mutations Associated with Non-familial Hypokalemic Periodic Paralysis

2011 ◽  
Vol 286 (31) ◽  
pp. 27425-27435 ◽  
Author(s):  
Chih-Jen Cheng ◽  
Shih-Hua Lin ◽  
Yi-Fen Lo ◽  
Sung-Sen Yang ◽  
Yu-Juei Hsu ◽  
...  
Keyword(s):  
Functional Characterization ◽  
Periodic Paralysis ◽  
Hypokalemic Periodic Paralysis

scholarly journals A Mutation in the KCNE3 Potassium Channel Gene Is Associated with Susceptibility to Thyrotoxic Hypokalemic Periodic Paralysis

2002 ◽  
Vol 87 (11) ◽  
pp. 4881-4884 ◽  
Author(s):  
Magnus R. Dias Da Silva ◽  
Janete M. Cerutti ◽  
Liliane A. T. Arnaldi ◽  
Rui M. B. Maciel
Keyword(s):  
Direct Sequencing ◽  
Genetic Defect ◽  
Sporadic Case ◽  
Ionic Channel ◽  
Periodic Paralysis ◽  
Resting Membrane Potential ◽  
Hypokalemic Periodic Paralysis ◽  
Dominant Disease ◽  
Periodic Paralyses ◽  
Thyrotoxic Hypokalemic Periodic Paralysis

Abstract Hypokalemic Periodic Paralyses comprise diverse diseases characterized by acute and reversible attacks of severe muscle weakness, associated with low serum potassium. The most common causes are Familial Hypokalemic Periodic Paralysis (FHypoKPP), an autosomal dominant disease, and Thyrotoxic Hypokalemic Periodic Paralysis (THypoKPP), secondary to thyrotoxicosis. Symptoms of paralysis are similar in both diseases, distinguished by thyrotoxicosis present in THypoKPP. FHypoKPP is caused by mutations in ionic channel genes calcium (CACN1AS), sodium (SCN4A) and potassium (KCNE3). Since both diseases are similar, we tested the hypothesis that THypoKPP could carry the same mutations described in FHypoKPP, being the paralysis a genetically conditioned complication of thyrotoxicosis. In 15 patients with THypoKPP, using target-exon PCR, CSGE screening, and direct sequencing, we excluded known mutations in CACN1AS and SCN4A genes. On the other hand, we were able to identify the R83H mutation in the KCNE3 gene in one sporadic case of THypoKPP, a man who had been asymptomatic until developing thyrotoxicosis caused by Graves’ disease; we confirmed the disease-causing mutation in 2 of 3 descendants. R83H was recently found in two FHypoKPP unrelated families, in which the mutant decreased outward potassium flux, resulting in a more positive resting membrane potential. We, therefore, identified the first genetic defect in THypoKPP, a mutation in the KCNE3 gene.

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