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Periodic Paralysis

Hypokalemic periodic paralysis


Presentation

  • I report a case of a 33-year-old Caucasian man who presented with sudden onset paralysis, following previous similar presentations without investigation. Blood tests revealed undetectable serum potassium levels in the context of paralysis.[ncbi.nlm.nih.gov]
  • We present the case of a 15-year-old Asian-American male who presented to a tertiary-care pediatric emergency department complaining of generalized weakness and flaccid paralysis of his lower extremities.[ncbi.nlm.nih.gov]
  • Here we present the case of a 25-year-old man who presented to the emergency department (ED) with sudden onset weakness of bilateral upper and lower extremities.[ncbi.nlm.nih.gov]
  • Therefore, it is premature to rule out TPP based on the presentation of high renal potassium secretion rates alone.[ncbi.nlm.nih.gov]
  • At presentation, he was found to have severe hypokalemia of 1.8 mEq/L. Further investigations led to the diagnosis of hyperthyroidism and Graves' disease.[ncbi.nlm.nih.gov]
Fishing
  • Some patients will do well with extra magnesium (the body's natural ion channel blocker ) or fish oil, while these same nutrients will make other patients worse.[en.wikipedia.org]
  • J Neurol Sci. 1996; 142 :126–33. [ PubMed : 8902732 ] Plaster NM, Tawil R, Tristani-Firouzi M, Canun S, Bendahhou S, Tsunoda A, Donaldson MR, Iannaccone ST, Brunt E, Barohn R, Clark J, Deymeer F, George AL Jr, Fish FA, Hahn A, Nitu A, Ozdemir C, Serdaroglu[ncbi.nlm.nih.gov]
Noncompliance
  • In this case, a 43-year-old African American woman with thyrotoxic periodic paralysis had recurrent lower extremity myopathy and acute respiratory failure precipitated by noncompliance with treatment for Graves disease.[ncbi.nlm.nih.gov]
  • The third was initially seen with hyperthyroidism and developed weakness and paralysis when his disease progressed because of therapeutic noncompliance.[ncbi.nlm.nih.gov]
Thyroid Nodule
  • We reported two cases of medically refractory THPP with recurrent paralysis of extremities and hypokalemia, and both were combined with thyroid nodules.[ncbi.nlm.nih.gov]
Recurrent Vomiting
  • A middle-aged man presented with episodes of transient quadriparesis preceded by recurrent vomiting.[ncbi.nlm.nih.gov]
Xerostomia
  • The patient, who had no xerostomia and xerophthalmia, was diagnosed with primary SS from serologic and histologic findings of minor salivary gland biopsy. The patient recovered after potassium replacement therapy.[ncbi.nlm.nih.gov]
Chvostek Sign
  • Chvostek's sign and Trousseau's sign were positive. Neurologically, she was fully oriented. She had Grade 3 power in all the four limbs with intact sensation.[ncbi.nlm.nih.gov]
Trousseau Sign
  • Chvostek's sign and Trousseau's sign were positive. Neurologically, she was fully oriented. She had Grade 3 power in all the four limbs with intact sensation.[ncbi.nlm.nih.gov]
Lower Limb Muscle Weakness
  • Neurological examination showed weakness in the muscles of the bilateral upper and lower limbs. Muscle weakness was more severe in the proximal site than in the distal site.[ncbi.nlm.nih.gov]
Inappropriate Laughter
  • One month later, the patient presented with dizziness and behavioral symptoms, such as inappropriate laughter and anger.[ncbi.nlm.nih.gov]
Anger
  • One month later, the patient presented with dizziness and behavioral symptoms, such as inappropriate laughter and anger.[ncbi.nlm.nih.gov]
Flaccid Paralysis of the Lower Extremity
  • We herein report a 25-yr-old Korean man who suffered from flaccid paralysis of the lower extremities and numbness of hands. The patient was subsequently diagnosed as having TPP associated with transient thyrotoxicosis due to painless thyroiditis.[ncbi.nlm.nih.gov]
  • We present the case of a 15-year-old Asian-American male who presented to a tertiary-care pediatric emergency department complaining of generalized weakness and flaccid paralysis of his lower extremities.[ncbi.nlm.nih.gov]

Workup

  • The patient was admitted to the intensive care unit where further workup revealed thyrotoxicosis and the patient was subsequently diagnosed with TPP.[ncbi.nlm.nih.gov]
  • Laboratory workup showed hypokalaemia, low thyroid-stimulating hormone and thyroxine (T4) but normal T3 and thyroglobulin levels. The radioiodine uptake scan also showed reduced uptake.[ncbi.nlm.nih.gov]
  • An association with thyroid disease is well established and further workup revealed Grave's disease in this patient.[casesjournal.biomedcentral.com]
Hypocalciuria
  • Documentation of hypocalciuria helps to differentiate GS from Bartter syndrome. We report a 44-year-old woman who presented with a history of seizure disorder and periodic paralysis.[ncbi.nlm.nih.gov]
  • Gitelman's syndrome is an autosomal recessive renal tubular disorder characterized by hypomagnesemia, hypokalemia, hypocalciuria, and metabolic alkalosis.[ncbi.nlm.nih.gov]
Right Bundle Branch Block
  • Our patient was found to have an extremely low serum potassium concentration, as well as an electrocardiogram that revealed a prolonged QT interval and right bundle branch block.[ncbi.nlm.nih.gov]

Treatment

  • Treatment includes the supplementation of potassium, a nonselective beta-blocker, and ultimately treatment of the underlying hyperthyroidism.[ncbi.nlm.nih.gov]
  • The patients experienced a decrease in the severity of their attacks upon initiation of treatment. Topiramate may warrant further investigation as a treatment option in hypoPP.[ncbi.nlm.nih.gov]
  • He rapidly recovered from weakness after acetazolamide treatment. Magnetic resonance imaging of thighs comparing pre- and post-treatment revealed a significant increase in muscle bulk.[ncbi.nlm.nih.gov]
  • Early diagnosis and appropriate treatment can avoid unnecessary investigations and serious cardiopulmonary complications.[ncbi.nlm.nih.gov]
  • Acute treatment of any electrolyte disorder, administration of a beta-blocker and treatment of the thyrotoxicosis leads to complete resolution.[ncbi.nlm.nih.gov]

Prognosis

  • OBJECTIVE: This review emphasizes the etiology, pathogenesis, management and prognosis of TPP and aims to highlight clinical awareness of early diagnosis and rapid initiation of treatment.[ncbi.nlm.nih.gov]
  • We present the clinical features and prognosis after adrenalectomy in this case. A 43-year-old Han Chinese male presented with severe hypokalaemic paralysis that remitted after taking oral potassium.[ncbi.nlm.nih.gov]
  • Antenatal diagnosis Prenatal diagnosis is theoretically possible if the disease-causing mutation in the family has been identified but is rarely performed because of the non life-threatening prognosis.[orpha.net]
  • Prognosis [ edit ] The prognosis for periodic paralysis varies.[en.wikipedia.org]
  • Prognosis Most patients learn to prevent their attacks well enough that no significant deterioration in the quality of life occurs. Strenuous exercise must be avoided, however. Attacks often lessen in severity and frequency during middle age.[encyclopedia.com]

Etiology

  • The etiology of these abnormalities and the patient's symptoms was found to be undiagnosed and uncontrolled hyperthyroidism from Grave's disease, which resulted in this dramatic presentation of thyrotoxic hypokalemic paralysis.[ncbi.nlm.nih.gov]
  • One of the challenges lies in recognizing the underlying etiology. Early diagnosis and appropriate treatment can avoid unnecessary investigations and serious cardiopulmonary complications.[ncbi.nlm.nih.gov]
  • OBJECTIVE: This review emphasizes the etiology, pathogenesis, management and prognosis of TPP and aims to highlight clinical awareness of early diagnosis and rapid initiation of treatment.[ncbi.nlm.nih.gov]
  • However, his serum potassium level rebounded rapidly within several hours after potassium supplementation, indicating that the intracellular shifting of potassium ions was the main etiology for his hypokalemia.[ncbi.nlm.nih.gov]
  • Other metabolic etiologies of vacuolar myopathy, such as acid phosphatase (lysosomal) associated acid maltase deficiency (a glycogen storage disease), need to be considered in the differential diagnosis.[ncbi.nlm.nih.gov]

Epidemiology

  • Summary Epidemiology Prevalence is estimated at around 1 in 100,000. Clinical description The paralysis most often affects the four limbs, thus resulting in tetraplegia.[orpha.net]
  • Summary Epidemiology The prevalence is estimated at around 1/200,000. Clinical description Attacks of muscle weakness generally begin during childhood (first decade).[orpha.net]
  • Epidemiology There are primary and secondary forms. The primary form is genetic and follows an autosomal dominant pattern of inheritance.[unboundmedicine.com]
  • Epidemiology Hypokalaemic periodic paralysis (HOKPP) has a prevalence of 1 case per 100,000 population.[patient.info]
  • Home, Search, Index, Links, Pathology, Molecules, Syndromes, Muscle, NMJ, Nerve, Spinal, Ataxia, Antibody & Biopsy, Patient Info EPISODIC MUSCLE WEAKNESS Hypokalemic Periodic Paralysis Hypokalemic periodic paralysis, Hereditary: General features Epidemiology[neuromuscular.wustl.edu]
Sex distribution
Age distribution

Pathophysiology

  • Here we report three cases of TPP in the Netherlands, and review the literature on clinical features, pathophysiological hypothesis and treatment.[ncbi.nlm.nih.gov]
  • This case report reviews the literature looking at pathophysiology, causes, and treatment recommendations for this rare complication of hyperthyroidism. [Indexed for MEDLINE] Free full text[ncbi.nlm.nih.gov]
  • Overview This article on periodic paralysis and related disorders introduces readers to the clinical presentations, basic concepts, and current understanding of the pathophysiology of these rare disorders or channelopathies.[medlink.com]
  • Further analysis regarding hormonal conditions and Kir channel expression are required to provide new clues about the TPP pathophysiology. Copyright 2017 IBRO. Published by Elsevier Ltd. All rights reserved.[ncbi.nlm.nih.gov]
  • Rüdel R (1986) The pathophysiological basis of the myotonias and the periodic paralyses. In: Engel AG, Banker BQ (eds) Myology, 1st edn. Mc-Graw-Hill, New York, pp 1297–1311 Google Scholar 12.[link.springer.com]

Prevention

  • It needs early recognition for proper management and prevention of recurrences.[ncbi.nlm.nih.gov]
  • Episodes can be prevented by achieving a euthyroid state. This report describes a young gentleman presenting with thyrotoxic hypokalemic paraparesis.[ncbi.nlm.nih.gov]
  • This case emphasises the importance of considering TPP in patients with acute muscle weakness and the importance of promptly initiating treatment and preventing relapse of TPP.[ncbi.nlm.nih.gov]
  • Early recognition by emergency physicians can prevent potential mortality. A 23-year-old Vietnamese male presented with a 2-h history of complete flaccid quadriplegia and chest tightness.[ncbi.nlm.nih.gov]
  • Therefore, it is necessary that early diagnosis of TPP due to transient thyrotoxicosis is made to administer definite treatment and prevent recurrent paralysis.[ncbi.nlm.nih.gov]

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