The clinical presentation of central pontine myelinolysis is variable. Patients may be asymptomatic, or have mild to severe symptoms, including rapid onset of pseudobulbar symptoms (e.g., difficulty swallowing or speaking), paraparesis, quadriparesis, confusion, psychiatric symptoms (e.g., delirium or hallucinations), "locked-in syndrome," in which there is paralysis of the lower limb musculature and cranial nerves resulting in a lack of expressive communication ability, although the intellectual activity is unaffected [1].

The symptoms may appear with a biphasic course, consisting of seizures and/or encephalopathy, with a period of improvement, followed by deterioration consisting of spastic quadriplegia, dysarthria, dysphagia, and occasionally oculomotor abnormalities such as horizontal gaze paralysis [2].

• Disability

  Severe disability (e.g., locked-in state) and high mortality were thought to be the usual outcomes in earlier reports (4). [doi.org]

  Here is a case of a disabled female who presented with polydipsia, polyuria, seizures, fetal death in utero, hyperglycemia, and hyper-osmolar hypernatremia on her 31st gestational week. [ncbi.nlm.nih.gov]

• Anemia

  An 87-year-old male, prescribed digoxin and furosemide for congestive heart failure and Alzheimer disease, had dehydration and anemia due to poor food intake and hemorrhagic cystitis. [ncbi.nlm.nih.gov]

  Investigations showed fractured neck of left femur with anemia (Hb 10.5 gm/dL), renal failure (blood urea 127 mg/dL, serum creatinine 5.1 mg/dL), metabolic acidosis (pH 7.3, pCO2 24.1 mmHg, HCO3 11.4 meq/dL, lactate 0.6 mmol/L), hypernatremia (147 meq [cjhr.org]

• Unconsciousness

  Unconsciousness and spastic paralysis of the limbs are the primary symptoms. Read More [britannica.com]

  Diffuse brain injury has been demonstrated in animal models to induce an immediate and prolonged post‐traumatic unconsciousness in the absence of mass lesions ( Gennarelli et al ., 1982 ). [doi.org]

• Dysphagia

  English central pontine myelinolysis demyelination disease that is characterized by severe damage to the myelin sheath of the pons' nerve cells and has symptom acute paralysis, has symptom dysphagia, and has symptom dysarthria central pontine myelinolysis [wikidata.org]

  We report a very case of a patient with dysarthria, dysphagia and psychiatric symptoms including abnormal behavior starting after alcohol withdrawal, with radiological evidence of CPM and EPM. [ncbi.nlm.nih.gov]

  Because of his dysphagia, he was kept on a Lavin tube and was treated with conservative care, including a thiamine supplement. [dx.doi.org]

• Progressive Dysphagia

  Case Report A 47-year-old man was admitted to the hospital with complaints of progressive dysphagia and dysarthria for a week, and this occurred just 2 days after he stopped drinking. His medical history was unremarkable. [dx.doi.org]

• Diplopia

  After two consecutive plasma exchange sessions, her neurologic symptoms were markedly improved except for mild diplopia. [ncbi.nlm.nih.gov]

  Relatively sudden onset of complex neurological symptoms including weakness, dysphagia, diplopia, disturbed consciousness and gait changes. Picture 1: Non enhanced CT of the brain. [southsudanmedicaljournal.com]

  CPM clinically presents with acute para- or quadraparesis, dysphagia, dysarthria, diplopia, loss of consciousness, and other neurological symptoms associated with brainstem damage. [cjhr.org]

  2015/18/1/84/144291 At 38-weeks gestation a 31-year-old primigravida presented with diplopia on left gaze of 1- day duration. The diplopia was worse for far objects. [annalsofian.org]

  Affectation of consciousness Changes in the gait Affectation of the respiratory function (decreased/ceased) Acute symptoms Paralysis (can affect two or all of the limbs) Difficulties swallowing (dysphagia) and speaking (dysarthria) Problems with the vision (diplopia [mddk.com]

• Encephalopathy

  Bovine spongiform encephalopathy. Lancet. 1990 Nov 24; 336 (8726):1300–1303. [ PubMed ] [ Google Scholar ] Collee JG. Bovine spongiform encephalopathy. [ncbi.nlm.nih.gov]

  Pontine reversible oedema: a newly recognized imaging variant of hypertensive encephalopathy? AJNR Am J Neuroradiol 2000 ; 21 : 243 –5. ↵ Kastrup O, Maschke M, Wanke I, et al. Posterior reversible encephalopathy syndrome due to severe hypercalcemia. [jnnp.bmj.com]

  In such a critically ill patient, it is likely that several factors contributed to his encephalopathy, including liver dysfunction, uremia, cyclosporine, and CPM. [jkms.org]

  Abstract We describe a non-alcoholic diabetic patient with central pontine myelinolysis (CPM) and Wernicke encephalopathy (WE). A 69-year-old man developed consciousness disturbance after parenteral hyperalimentation for liver abscess and sepsis. [doi.org]

• Dysarthria

  There was little improvement in the dysarthria or psychiatric symptoms in the first month. [ncbi.nlm.nih.gov]

  Our patient presented initially with the symptoms of CPM, exhibiting lethargy, quadriparesis, and dysarthria. [movementdisorders.org]

  He only displayed bulbar palsy and dysarthria. [dx.doi.org]

• Altered Mental Status

  Although her bicarbonate deficit was 365 mEq, we treated her with an overdose of intravenous sodium bicarbonate, 480 mEq for 24 hours, due to the severity of her acidemia and her altered mental status. [ncbi.nlm.nih.gov]

  We report a case of osmotic demyelination syndrome in a 55-year-old female, with a past medical history of arterial hypertension and multi-level cervical spondylosis, hospitalized for acute altered mental status complicating an array of acute gastroenteritis [scienceopen.com]

  Some of the signs and symptoms include: Difficulty in speaking Difficulty in swallowing Double vison Muscle cramps Convulsions Hallucinations Poor reflexes Paralysis of various parts of the body Loss of sensation in various parts of the body Altered mental [dovemed.com]

• Neurologic Manifestation

  Central pontine myelinolysis should be included in the differential diagnosis when neurologic manifestations are observed after living donor liver transplantation. [ncbi.nlm.nih.gov]

  Three to five days later, a second phase of neurological manifestations occurs correlating with the onset of myelinolysis. [en.wikipedia.org]

• Lethargy

  A 26-year-old man with a history of chronic alcohol abuse presented with dysarthria, lethargy, and horizontal nystagmus. [dx.doi.org]

  Common symptoms included lethargy and dysarthria. Five of the 6 patients had chronic alcoholism. All had improvements in their clinical conditions upon hospital discharge. [ncbi.nlm.nih.gov]

  Our patient presented initially with the symptoms of CPM, exhibiting lethargy, quadriparesis, and dysarthria. [movementdisorders.org]

  Radiation treatment of the brain Severe nausea and vomiting during pregnancy Symptoms Symptoms may include any of the following: Confusion, delirium, hallucinations Balance problems, tremor Problem swallowing Reduced alertness, drowsiness or sleepiness, lethargy [leehealth.org]

Workup of central pontine myelinolysis consists of a history, physical exam, laboratory testing, and imaging. Patients with a history of rapid correction of severe hyponatremia (plasma serum level <120 mq/L persisting for two or more days, that has been corrected at a rate greater than 8 mq/L in 48 hours), patients with liver disease or alcohol abuse should be considered at high-risk for central pontine myelinolysis [3] [4]. During the neurologic exam, the clinician should assess signs of confusion, occulomotor dysfunction (e.g., horizontal gaze paralysis), impaired speech or swallowing, and signs of spastic quadriplegia such as limb weakness, increased tonicity of the limb muscles, and hyperreflexia). The primary laboratory test consists of a chemistry panel (sodium, potassium, chloride, bicarbonate, glucose, creatine, and BUN). Liver function test can also be performed when clinically indicated.

Computed tomography (CT) and magnetic resonance imaging (MRI) have been used to aid in the diagnosis [5] [6] [7] [8]. Imaging test results may be normal during the early stages of the disease (through two weeks); after this period imaging abnormalities indicative of central pontine myelinolysis emerge. MRI is more sensitive for diagnosing abnormalities than CT scan [9] [10]. Comparison of changes between earlier and later CT or MRI scans is also useful.

CT scan images are prone to streak artifact in the region of the pons (and thus not as sensitive as MRI). CT scan may reveal low attenuation crossing the midline in the lower pons.

On T2-weighted MRI scans, areas of demyelination are marked by hyperintense and bright areas. T1 signal may be mildly or moderately hypointense; fluid attenuation inversion recovery (FLAIR) signal will usually appear hyperintense. A classic trident shaped pattern is indicative of ventrolateral pons, corticospinal tract, and tegmentum sparing. Extrapontine lesions are usually bilateral; they are commonly found on the cerebellar peduncles, globus pallidus, lateral geniculate body, thalamus, and putamen [5] [11].

Cerebral spinal fluid evaluation may show an elevated opening pressure, mononuclear pleocytosis, or an elevated protein level.

Few advanced neuroimaging studies like positron emission tomography have been studied for its efficacy in the diagnosis of central pontine myelinosis; studies have shown a high FDG-18 uptake initially, followed by decreased uptake in affected regions [12].

Electroencephalography (EEG) can be used to supplement imaging results, particularly when neuroimaging is not definitive. EEG usually reveals diffuse bihemispheric slowing in patients with central pontine myelinolysis.

• Hypophosphatemia

  We report an unusual case of CPM likely due to hypophosphatemia, related to a refeeding syndrome in the context of mental anorexia. [dx.doi.org]

  Other causes include cirrhosis, severe burns, hypokalemia, hyperosmolar hyperglycemic state, acquired immunodeficiency syndrome (AIDS), post-pituitary surgery, post-urological surgery, psychogenic polydipsia, beer potomania, prolonged diuretic use, hypophosphatemia [cjhr.org]

  Conditions associated with CPM include chronic alcoholism (40%), rapid correction of hyponatremia (20%), postliver-transplantation (17%), cirrhosis (5%) and hyperglycemia, azotemia, hypernatremia, and rapid normalization of hypophosphatemia. [ijam-web.org]

  Etiology of Pontine Myelinolysis Causes Reference Liver transplant [44,48-50] Alcohol [45,51] Hypophosphatemia [52] Type 1 diabetes [53] Gestational diabetes [54] Hypernatremia [55,56] Wilson’s disease [46] Hyperglycemia [55,57] Celiac disease [8] Primary [transbiomedicine.com]

• Hypophosphatemia

  We report an unusual case of CPM likely due to hypophosphatemia, related to a refeeding syndrome in the context of mental anorexia. [dx.doi.org]

  Other causes include cirrhosis, severe burns, hypokalemia, hyperosmolar hyperglycemic state, acquired immunodeficiency syndrome (AIDS), post-pituitary surgery, post-urological surgery, psychogenic polydipsia, beer potomania, prolonged diuretic use, hypophosphatemia [cjhr.org]

  Conditions associated with CPM include chronic alcoholism (40%), rapid correction of hyponatremia (20%), postliver-transplantation (17%), cirrhosis (5%) and hyperglycemia, azotemia, hypernatremia, and rapid normalization of hypophosphatemia. [ijam-web.org]

  Etiology of Pontine Myelinolysis Causes Reference Liver transplant [44,48-50] Alcohol [45,51] Hypophosphatemia [52] Type 1 diabetes [53] Gestational diabetes [54] Hypernatremia [55,56] Wilson’s disease [46] Hyperglycemia [55,57] Celiac disease [8] Primary [transbiomedicine.com]

• Hypoglycemia

  Central pontine myelinolysis following hypoglycemia. CHRISMED J Health Res 2014;1:214-5 How to cite this URL: Demel R, George P, George U, Pandian J. Central pontine myelinolysis following hypoglycemia. [cjhr.org]

  A case of asymptomatic central pontine myelinolysis after severe hypoglycemia in a patient with diabetic nephropathy. Korean J Nephrol 2005;24:300-4. 19. Burns JD, Kosa SC, Wijdicks EF. [jnaccjournal.org]

Central pontine myelinolysis occurs most often as a complication of treatment of patients with profound, life-threatenin-g hyponatremia (low sodium) and is a consequence of a rapid rise in serum tonicity following treatment in individuals with chronic [physio-pedia.com]

The aetiology is not clear, and there is no evidence regarding the optimal treatment or prognosis of CPM. [ncbi.nlm.nih.gov]

In general, this neurological disorder has a fatal prognosis. We observed a 30-year-old woman with a history of chronic alcohol abuse but without evidence of hyponatremia, who developed severe pontine central myelinolysis. [ncbi.nlm.nih.gov]

We aimed to describe the baseline characteristics, the management, the long-term prognosis, and the prognostic factors in central or extrapontine myelinolysis. [dx.doi.org]

We cannot explain the reason and mechanism of the poor prognosis in this case, although we could predict a poor prognosis from the serial MRIs. [alcalc.oxfordjournals.org]

When an uncommon etiology for CPM is suspected, exhaustive workup, including evaluation for autoimmune etiologies, should occur and guide management prior to prognostication. These patients may be responsive to steroids. [hoajonline.com]

The etiology of central pontine myelinolysis (CPM) is usually related to rapid correction of hyponatremia and alcoholism. [ncbi.nlm.nih.gov]

Definition / general Central pontine and extrapontine myelinolysis are complications of treatment of marked hyponatremia Terminology Also termed osmotic demyelination syndrome Epidemiology Occurs most commonly in setting of chronic alcoholism (~40% of [pathologyoutlines.com]

Sandrine Leroy 1 Department of Epidemiology of Emerging Diseases Unit, Insititut Pasteur, Paris, France Ariel Gout 2 Department of Neuropediatrics, Bicetre Hospital, Paris, France, Beatrice Husson 3 Department of Pediatric Neurology, Bicêtre University [dx.doi.org]

Epidemiology The exact incidence of central pontine myelinolysis is unknown. A study by Singh et al demonstrated that central pontine myelinolysis was present in 29% of postmortem examinations of liver transplant patients. [emedicine.com]

The pathophysiology of CPM has not been well understood. [ajnr.org]

We also discuss factors that may contribute to the development of central pontine myelinolysis after orthotopic liver transplant and its pathophysiology. [ncbi.nlm.nih.gov]

The morbidity and mortality of CPM/EPM has been greatly reduced by recognition of pre-disposing conditions, increased understanding of the pathophysiology, intensive treatment, and rapid diagnosis and monitoring with advanced neuroimaging. [dx.doi.org]

Prevention Slow correction of electrolytic imbalance. central pontine myelinolysis Neuropathology A condition characterized by softening of the base of the brain at the pons with damage to the myelin sheath, related to aggressive correction of hyponatremia [medical-dictionary.thefreedictionary.com]

The appropriate administration of thiamine may prevent poor outcomes. [ncbi.nlm.nih.gov]

Prevention and Treatment The most certain way to prevent central pontine myelinolysis is to correct the reduced sodium levels in a gradual manner. [mddk.com]

Prevention and treatment of pontine myelinolysis Several options surround the prevention and treatment of PM. A hallmark of prevention is proper management of hyponatremia post-absorption of irrigation fluid. [transbiomedicine.com]

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