Presentation
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]
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]
Recurrent hypokalemic paralysis is an extremely unusual presentation of hypothyroidism. [ijem.in]
Paramyotonia congenita (Online Mendelian Inheritance in Man (OMIM) 168300), a form which often accompanies hyperkalemic periodic paralysis, but may present alone. [en.wikipedia.org]
Cytoplasmic bodies were present. No tubular aggregates could be found. The nerve bundles were normal. These findings were in part similar to the ones reported by Gérard et al. (1978) in the son's biopsy during an induced paralytic attack. [ncbi.nlm.nih.gov]
Entire Body System
- 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]
- Heat Intolerance
Symptoms of thyrotoxicosis (eg, anxiety, emotional lability, weakness, tremor, palpitations, heat intolerance, increased perspiration, weight loss) are typically present. [msdmanuals.com]
People have symptoms of hyperthyroidism, such as anxiety, tremors, palpitations, and heat intolerance. In Andersen-Tawil syndrome, attacks of weakness usually begin before age 20 and are triggered by rest after exercise. [merckmanuals.com]
- Intravenous Administration
The following can cause hypokalemia Alcoholism Hypokalemic attack may be precipitated by the administration of oral glucose, 1.5g/kg body weight (up to 100 g) Intravenous administration of insulin, maximum 0.1 U/kg body weight at 30 and 60 minutes, during [alternativehealing.org]
- Inguinal Hernia
Case Report The patient was a 53-yr-old man who was seen at our Preoperative Evaluation Center the day prior to undergoing radical retropubic prostatectomy and right inguinal hernia repair. [anesthesiology.pubs.asahq.org]
Gastrointestinal
- Eating Frequently
People can prevent attacks by eating frequent meals rich in carbohydrates and low in potassium and by avoiding fasting, strenuous activity after meals, and exposure to cold. [merckmanuals.com]
Ears
- Low Set Ears
Patients with ATS may also have skeletal abnormalities like scoliosis (curvature of the spine), webbing between the second and third toes or fingers (syndactyly), crooked fingers (clinodactyly), a small jaw (micrognathia) and low-set ears. [en.wikipedia.org]
Episodes usually begin before age 20 with all or some of the clinical triad: Periodic paralysis Prolonged QT interval and ventricular arrhythmias Dysmorphic physical features Dysmorphic physical features include short stature, high-arched palate, low-set [msdmanuals.com]
People with this form may also have other disorders such as an abnormally curved spine (scoliosis), webbed fingers and toes (syndactyly), abnormally positioned fingers and toes (clinodactyly), a small chin (micrognathia), or low-set ears. [merckmanuals.com]
Musculoskeletal
- Myopathy
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]
(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]
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]
Neurologic
- Flaccid Paralysis
Definition (MSH) A heterogenous group of inherited disorders characterized by recurring attacks of rapidly progressive flaccid paralysis or myotonia. [fpnotebook.com]
[…] 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]
Workup
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]
Treatment
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]
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]
Treatment depends on the form. Each form of familial periodic paralysis involves a different gene and electrolyte channel. [msdmanuals.com]
Participants will be randomly assigned to one of two treatment groups: dichlorphenamide or placebo (an inactive substance). [clinicaltrials.gov]
Prognosis
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]
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 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]
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]
Etiology
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]
Epidemiology
(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]
Pathophysiology
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;10:143-8. 3. Lin SH, Lin YF, Halperin ML. Hypokalemia and paralysis. QJM 2001;94:133-9. 4. [annalsofian.org]
Prevention
Prevention There is no way to prevent the occurrence of either disease in a person with the gene for the disease. [encyclopedia.com]
Treat episodes by correcting serum potassium and prevent episodes by recommending lifestyle changes. Click here for Patient Education [msdmanuals.com]
To prevent attacks, doctors give people drugs to keep their thyroid gland functioning properly and beta-blockers (such as propranolol). [merckmanuals.com]