A case of a 29 year old male is presented here. The patient presented with sudden onset paralysis of his extremities. Laboratory evaluation revealed a markedly low potassium level. [casesjournal.biomedcentral.com]

The theoretical analysis presented here shows that although such hypotheses are qualitatively correct, the quantitative contribution of the electrogenic pump is too small to account entirely for the changes in membrane potential observed between the paralytic [ncbi.nlm.nih.gov]

The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment. [orpha.net]

Age It tends to present in the second decade of life but may be in the first decade with more severe disease and the third decade in milder cases. The majority present before 16 years of age. [patient.info]

The present study was undertaken to know the etiology of hypokalemic paralysis, its varied presentations, and the clinical outcome after potassium replacement therapy of this easily treatable metabolic disorder. [annalsofian.org]

• Fatigue

  Factors that can trigger attacks include rest after exercise, potassium-rich foods such as bananas and potatoes, stress, fatigue, alcohol, pregnancy, exposure to hot or cold temperatures, certain medications, and periods without food (fasting). [ghr.nlm.nih.gov]

  Factors that can trigger attacks include rest after exercise, potassium-rich foods such as bananas and potatoes, stress, fatigue, alcohol, pregnancy, exposure to cold temperatures, certain medications, and periods without food (fasting). [icdlist.com]

  Myopathy : This form is characterized by permanent muscle weakness, fatigue, and pain. More than 74 percent of older people with hypoPP experience myopathy. [healthline.com]

  The paralytic form is more common, causing randomly spaced attacks (or episodes) of weakness which range from mild (which feels like fatigue) to flaccid (rag-doll-type) paralysis triggered by a fall in serum potassium. [hkpp.org]

  Attacks may be provoked by stress such as a viral illness or fatigue, or certain medications such as beta-agonists, insulin or steroids. A perturbation of sodium and calcium ion channels results in low potassium levels and muscle dysfunction [ 2 ]. [casesjournal.biomedcentral.com]

• Diarrhea

  Appearance of symptoms is typically in the first or second decade of life (60% of cases have onset aged < 16 years) with susceptible individuals experiencing sudden onset of perioral numbness; weakness; centrifugal paralysis, often with nausea; vomiting and diarrhea [mdedge.com]

  Crash diets with inadequate intake of potassium Chronic stress which increases adrenocortical hormone Chronic diarrhea, mal-absorption syndrome Perspiration and chronic fever Renal tubular acidosis - primary Renal tubular acidosis - secondary to amphotericin [alternativehealing.org]

  Página 54 - Pedigree demonstrating a sex-linked recessive condition characterized by draining ears, eczematoid dermatitis and bloody diarrhea. ‎ Página 51 - Selective IgA deficiency: presentation of 30 cases and a review of the literature. ‎ [books.google.es]

  Prior to this episode, the patient had been healthy and denied any recent diarrhea, chest pain, shortness of breath, or weight change. He did report several episodes of waking from sleep with a "racing heart." [casesjournal.biomedcentral.com]

  […] doctor or other medical specialist, for example, a neurologist, will likely run some preliminary tests to rule in or rule out (called a differential diagnosis) other causes of muscle weakness such as licorice intoxication, laxative abuse, and/or severe diarrhea [medicinenet.com]

• Nausea

  […] inexcitable. 2 Appearance of symptoms is typically in the first or second decade of life (60% of cases have onset aged < 16 years) with susceptible individuals experiencing sudden onset of perioral numbness; weakness; centrifugal paralysis, often with nausea [mdedge.com]

  My potassium has been as low as 2.1 during an attack and it causes nausea and vomiting. I have been placed in Critical Care while in the hospital to get my attack under control. [globalgenes.org]

  My potassium has been as low as 2.1 during an attack and it causes nausea and vomiting. I have been placed in critical care while in the hospital to get my attack under control. [themighty.com]

  The absence of polyuria, polydipsia, nausea, vomiting, constipation, hypochloremia, and hyponatremia ruled out Bartter syndrome. Normal serum magnesium and urinary calcium excretion ruled out the possibility of Gitelman's syndrome. [ijem.in]

  If no improvement is apparent after four to five oral doses, or if nausea or diarrhea occurs after oral KCl intake, IV administration of KCl is necessary. [hkpp.org]

• Vomiting

  […] inexcitable. 2 Appearance of symptoms is typically in the first or second decade of life (60% of cases have onset aged < 16 years) with susceptible individuals experiencing sudden onset of perioral numbness; weakness; centrifugal paralysis, often with nausea; vomiting [mdedge.com]

  My potassium has been as low as 2.1 during an attack and it causes nausea and vomiting. I have been placed in Critical Care while in the hospital to get my attack under control. [globalgenes.org]

  The absence of polyuria, polydipsia, nausea, vomiting, constipation, hypochloremia, and hyponatremia ruled out Bartter syndrome. Normal serum magnesium and urinary calcium excretion ruled out the possibility of Gitelman's syndrome. [ijem.in]

  My potassium has been as low as 2.1 during an attack and it causes nausea and vomiting. I have been placed in critical care while in the hospital to get my attack under control. [themighty.com]

  Table 2 Causes of Hypokalemia Increased aldosterone Diuretics Hypomagnesemia Renal Tubular Acidosis (Type I and II) Metabolic alkalosis Liddle's syndrome Potassium Depletion – Extra-renal Decreased intake Vomiting/Diarrhea Zollinger-Ellison Syndrome Fistulas [casesjournal.biomedcentral.com]

• Myopathy

  (s): Inflammatory myopathy NOS G72.8 Other specified myopathies G72.81 Critical illness myopathy Inclusion term(s): Acute necrotizing myopathy Acute quadriplegic myopathy Intensive care (ICU) myopathy Myopathy of critical illness G72.89 Other specified [icd10coded.com]

  G71.3 Mitochondrial myopathy, not elsewhere classified G71.8 Other primary disorders of muscles G71.9 Primary disorder of muscle, unspecified G72 Other and unspecified myopathies G72.0 Drug-induced myopathy G72.1 Alcoholic myopathy G72.2 Myopathy due [icd10data.com]

  The myopathy associated with hypokalemic familial periodic paralysis is thus another cause for the presence of CK 2 (MB). [ncbi.nlm.nih.gov]

  Myopathy : This form is characterized by permanent muscle weakness, fatigue, and pain. More than 74 percent of older people with hypoPP experience myopathy. [healthline.com]

• Myalgia

  Atypical periodic paralysis and myalgia: A novel RYR1 phenotype. Neurology. 2018 Jan 30; 90(5):e412-e418. [invitae.com]

  […] over years Some patients Muscle rigidity Calf hypertrophy Penetrance: Male 100%; Female 50% Clinical features with specific mutations Arg528His 4 Common Later onset: Mean 14.5 years in males; 11.8 years in females Reduced penetrance in females (50%) Myalgias [neuromuscular.wustl.edu]

  All the 3 patients had severe myalgia, and they took longer time to recover in comparison to other secondary disorders. [annalsofian.org]

• Muscle Spasm

  Muscle spasms are common. [raredr.com]

  Note: Because the generalized muscle spasms associated with such attacks may lead to an increase in body temperature, individuals with hyperPP have been considered to be susceptible to malignant hyperthermia. [ncbi.nlm.nih.gov]

• Flaccid Paralysis

  […] diseases characterized by episodic flaccid paralysis or muscular weakness. progressive bulbar paralysis the chronic form of bulbar paralysis; called also Duchenne's disease or paralysis. [medical-dictionary.thefreedictionary.com]

  […] disorders characterized by recurring attacks of rapidly progressive flaccid paralysis or myotonia; these conditions have in common a mutation of the gene encoding the alpha subunit of the sodium channel in skeletal muscle; frequently associated with [icd9data.com]

  Definition (MSH) A heterogenous group of inherited disorders characterized by recurring attacks of rapidly progressive flaccid paralysis or myotonia. [fpnotebook.com]

An association with thyroid disease is well established and further workup revealed Grave's disease in this patient. [casesjournal.biomedcentral.com]

Cardiac evaluations using serial ECGs with measurements of the QTc interval are essential and should be performed on all patients undergoing workup for periodic paralysis. 23, 24, 25 References 1. [hkpp.org]

• Prominent A-Wave

  Prolongation of the PR and QT intervals and T-wave flattening are associated with prominent U-waves. [hkpp.org]

  SCN4A (20%) External link: YouTube video Laboratory Serum K + : Low during attacks Serum CK: Often elevated during attacks NCV & EMG during attacks CMAP amplitudes: Small Insertional activity: Reduced Spontaneous activity: Fibrillations; Positive sharp waves [neuromuscular.wustl.edu]

Patients often report years of wrong diagnosis and treatments that made them worse instead of better. [en.wikipedia.org]

References 1.Taro’s Keveyis™ (dichlorphenamide) 50 mg Tablets Now Available for the Treatment of Primary Hyperkalemic and Hypokalemic Periodic Paralysis [news release]. [raredr.com]

Participants will be randomly assigned to one of two treatment groups: dichlorphenamide or placebo (an inactive substance). [clinicaltrials.gov]

Follow-up studies proved that the inhalation of salbutamol is a simple and adequate method for the treatment of the paralytic episodes in these patients. [ncbi.nlm.nih.gov]

The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment. [orpha.net]

Family history Timing Intensity History of administration of certain drugs Clinical examination Differential Diagnosis Laboratory investigations Serum K+ CPK and serum myoglobin ECG EMG Nerve conduction studies Provocative Testing Muscle biopsy Treatment Prognosis [slideshare.net]

Severe prognosis in a large family with hypokalemic periodic paralysis. Muscle Nerve. 2003;27:165–9. 13. Mutation analysis of two patients with hypokalemic periodic paralysis and suspected malignant hyperthermia. Muscle Nerve. 2004;30:114–7. [revistas.fucsalud.edu.co]

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]

Severe prognosis in a large family whit hypokalemic periodic paralysis. Muscle Nerve 2003;27:165-9. Fontaine B, Vale Santos J, Jurkat-Rott K, Reboul J, et al. [medigraphic.com]

Literatur Caciotti A, Morrone A, Domenici R, Donati MA, Zammarchi E (2003) Severe prognosis in a large family with hypokalemic periodic paralysis. [link.springer.com]

Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology. [icd10coded.com]

Follow up studies were performed to determine the etiology of the patient's hypokalemia. Urine sodium and potassium, and serum aldosterone and renin levels were measured to rule out adrenal involvement and were found to be normal. [casesjournal.biomedcentral.com]

[…] reported to be associated with hypokalemic paralysis. 3 Related Long-Term Surgical Management of Severe Pelvic Injury and Resulting Neurogenic Bladder From an Improvised Explosive Device Pa Ping, a condition involving hypokalemic paralysis of uncertain etiology [mdedge.com]

Hypokalemic periodic paralysis though common among Indian population varies greatly in disease spectrum and magnitude in our country due to the heterogeneous pattern of etiology behind it. [ijem.in]

Objective: To study the etiology, varied presentations, and outcome after therapy of patients with hypokalemic paralysis. [annalsofian.org]

(including hypokalemic periodic paralysis and thyrotoxic periodic paralysis, and other “Channelopathies” or “Membranopathies) Pathophysiology Epidemiology Primary or familial periodic paralysis Secondary periodic paralysis Conventional classification [slideshare.net]

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]

Epidemiology Hypokalaemic periodic paralysis (HOKPP) has a prevalence of 1 case per 100,000 population. [patient.info]

This review focuses on the clinical features, molecular genetic defects, and pathophysiologic mechanisms that underlie familial periodic paralysis. [ncbi.nlm.nih.gov]

(including hypokalemic periodic paralysis and thyrotoxic periodic paralysis, and other “Channelopathies” or “Membranopathies) Pathophysiology Epidemiology Primary or familial periodic paralysis Secondary periodic paralysis Conventional classification [slideshare.net]

In this chapter, we describe the pathophysiology, clinical presentation, laboratory findings, and treatment of the nondystrophic myotonias and periodic paralyses ( Table 31-1 ). [neurology.mhmedical.com]

Br J Anaesth 1980; 52: 153–64Ellis, FR Miller J, Katz RL: Muscle diseases, Anesthesia and Uncommon Disease: Pathophysiologic and Clinical Correlations, 2nd edition. Edited by Katz J, Benumof J, Kadis LB. [anesthesiology.pubs.asahq.org]

Hypokalemic paralyses: a review of the etiologies, pathophysiology, presentation and therapy. Am J Emerg Med 1992;10:143-6. Kruse BA, Carlson RW. Rapid correction of hipokalemia using concentrated intravenous potassium chloride infusions. [medigraphic.com]

Treat episodes by correcting serum potassium and prevent episodes by recommending lifestyle changes. Click here for Patient Education [msdmanuals.com]

Treat episodes by correcting serum potassium and prevent episodes by recommending lifestyle changes. [merckmanuals.com]

Taking potassium supplements may help prevent muscle weakness. Eating a low-carbohydrate diet may help decrease symptoms. A medicine called acetazolamide may be prescribed to prevent attacks. [medlineplus.gov]

Prevention There is no way to prevent the occurrence of either disease in a person with the gene for the disease. [encyclopedia.com]