Consequences of Hyponatremia: Central Pontine and Extrapontine Myelinolysis in a Chronic Alcohol User.

Central pontine myelinolysis (CPM) and extrapontine myelinolysis (EPM) are rare neurological disorders associated with rapid correction of hyponatremia, particularly in individuals with chronic alcohol use. We present the case of a 52-year-old male with a history of chronic alcoholism who developed CPM and EPM following correction of severe hyponatremia. The patient presented with dysarthria, hemiparesis, and altered mental status, which progressed rapidly to pseudobulbar features and spastic quadriparesis. Neuroimaging revealed characteristic findings of CPM and EPM. Treatment with intravenous dexamethasone, intravenous immunoglobulin (IVIG), and methylprednisolone led to gradual neurological improvement. The patient showed significant recovery after two months, highlighting the importance of early recognition and cautious management of electrolyte disturbances in high-risk individuals to prevent devastating neurological complications.

Central Pontine Myelinolysis: A Rare and Life-Threatening Adverse Effect of Clobazam and Quetiapine.

Central pontine myelinolysis (CPM) is a rare neurological disorder characterized by demyelination within the central portion of the pons. While hyponatremia is a well-known precipitating factor, other etiologies, including medication use, have been reported. We present a case of a 69-year-old male with a history of obsessive-compulsive disorder, stroke, and type 2 diabetes mellitus who developed confusion, altered sensorium, and weakness in all four limbs. An MRI brain imaging revealed characteristic findings suggestive of CPM. Despite normal serum sodium levels, discontinuation of clobazam and quetiapine, medications taken by the patient, led to clinical improvement. This case underscores the importance of considering medication-induced CPM in the differential diagnosis of patients presenting with neurological symptoms, even in the absence of electrolyte abnormalities.

Pontine myelinolysis with an uncommon manifestation of hallucination: a case report.

This report aims to present an elderly woman with persistent delirium after hospitalization for lethargy secondary to hyponatremia. The diagnosis of pontine myelinolysis was made and there were no characteristic neurological manifestations such as pupillary changes or spastic tetraparesis. Hallucinations and personality changes were the clues to the diagnosis and should be considered an atypical manifestation of pontine myelinolysis.

O objetivo deste relato é apresentar uma idosa que apresentou, após internação por letargia secundária à hiponatremia, delirium persistente depois da alta hospitalar. O diagnóstico de mielinólise pontina foi feito após a alta hospitalar e não houve manifestações neurológicas características, como alterações pupilares ou tetraparesia espástica. Alucinações e mudança de personalidade foram as pistas para o diagnóstico e devem ser consideradas como uma manifestação atípica de mielinólise pontina.

Unraveling Neurological Shades: Vitamin D Toxication and Central Pontine Myelinolysis Exposed.

This case report is about a middle-aged female who presented with complaints of pain in the abdomen with intractable vomiting for three months, pain and weakness in bilateral lower limbs for two months, and irritability for three days. She was previously treated for lumbar disc bulge and severe narrowing of the spinal cord whose treatment also included vitamin D supplements. After taking high doses of a vitamin D supplement daily for approximately four months, it resulted in vitamin D toxicity. The sodium level of the patient was in the normal range throughout the treatment. Her magnetic resonance imaging brain revealed features of central pontine myelinolysis. The development of central pontine myelinolysis due to vitamin D toxicity, with normal sodium levels, makes this a rare case for discussion.

Osmotic demyelination as a complication of hyponatremia correction: a systematic review / Desmielinização osmótica como complicação da correção de hiponatremia: uma revisão sistemática

ABSTRACT Background: Rapid correction of hyponatremia, especially when severe and chronic, can result in osmotic demyelination. The latest guideline for diagnosis and treatment of hyponatremia (2014) recommends a correction limit of 10 mEq/L/day. Our aim was to summarize published cases of osmotic demyelination to assess the adequacy of this recommendation. Method: Systematic review of case reports of osmotic demyelination. We included cases confirmed by imaging or pathology exam, in people over 18 years of age, published between 1997 and 2019, in English or Portuguese. Results: We evaluated 96 cases of osmotic demyelination, 58.3% female, with a mean age of 48.2 ± 12.9 years. Median admission serum sodium was 105 mEq/L and > 90% of patients had severe hyponatremia (<120 mEq/L). Reports of gastrointestinal tract disorders (38.5%), alcoholism (31.3%) and use of diuretics (27%) were common. Correction of hyponatremia was performed mainly with isotonic (46.9%) or hypertonic (33.7%) saline solution. Correction of associated hypokalemia occurred in 18.8%. In 66.6% of cases there was correction of natremia above 10 mEq/L on the first day of hospitalization; the rate was not reported in 22.9% and in only 10.4% was it less than 10 mEq/L/day. Conclusion: The development of osmotic demyelination was predominant in women under 50 years of age, with severe hyponatremia and rapid correction. In 10.4% of cases, there was demyelination even with correction <10 mEq/L/day. These data reinforce the need for conservative targets for high-risk patients, such as 4-6 mEq/L/day, not exceeding the limit of 8 mEq/L/day.

RESUMO Antecedentes: A correção rápida da hiponatremia, principalmente quando grave e crônica, pode resultar em desmielinização osmótica. A última diretriz para diagnóstico e tratamento da hiponatremia (2014) recomenda um limite de correção de 10 mEq/L/dia. Nosso objetivo foi sumarizar os casos publicados de desmielinização osmótica para avaliar a adequação dessa recomendação. Método: Revisão sistemática de relatos de caso de desmielinização osmótica. Incluímos casos confirmados por imagem ou anatomia patológica, em maiores de 18 anos, publicados entre 1997 e 2019, nas línguas inglesa ou portuguesa. Resultados: Avaliamos 96 casos de desmielinização osmótica, sendo 58,3% do sexo feminino e com média de idade de 48,2 ± 12,9 anos. A mediana de sódio sérico admissional foi 105 mEq/L e > 90% dos pacientes apresentavam hiponatremia grave (<120 mEq/L). Foram comuns os relatos de distúrbios do trato gastrointestinal (38,5%), etilismo (31,3%) e uso de diuréticos (27%). A correção da hiponatremia foi feita principalmente com solução salina isotônica (46,9%) ou hipertônica (33,7%). Correção de hipocalemia associada ocorreu em 18,8%. Em 66,6% dos casos houve correção da natremia acima de 10 mEq/L no primeiro dia de internamento; a velocidade não foi relatada em 22,9% e em apenas 10,4% foi menor que 10 mEq/L/dia. Conclusão: O desenvolvimento da desmielinização osmótica foi predominante em mulheres, abaixo de 50 anos, com hiponatremia grave e correção rápida. Em 10,4% dos casos, houve desmielinização mesmo com correção <10 mEq/L/dia. Esses dados reforçam a necessidade de alvos conservadores para pacientes de alto risco, como 4-6 mEq/L/dia, não ultrapassando o limite de 8 mEq/L/dia.

Symptomatic and asymptomatic pontine hyperintensities: are they central pontine myelinolysis? Case series.

The diagnosis of central pontine hyperintensities on cranial magnetic resonance imaging has wide-spectrum clinical entities, and the pro-type diagnosis of these is central pontine myelinolysis. The rapid changes in extracellular environment due to an imbalance of electrolytes or metabolic or toxic stress to the myelin sheath can cause demyelination of white matter tracts in pons, which is why this situation could have different etiologies than just rapid sodium changes. Central pontine myelinolysis can be presented with different clinical scenarios from asymptomatic to death. Here, we present eight patients who had central pontine hyperintensities on radiological imaging but showed various clinical symptoms and different etiologies. The etiologies of these cases are hypertriglyceridemia, alcohol consumption, antidepressant usage, immunocompromised situations, uncontrolled diabetes mellitus, liver and kidney failure, electrolyte changes, and adrenal insufficiency. We aimed to take attention with this case series to the fact that not only fast correction of hyponatremia is the cause of central pontine myelinolysis, but also different etiologies could play a role in this situation, and making the central pontine myelinolysis diagnosis considering different diagnosis is very important.

Rhabdomyolysis Complicated with Hyponatremia Due to Water Intoxication and Severe Aspiration Pneumonia: Description of a Patient with Chronic Schizophrenia and Literature Review.

Hyponatremia due to water intoxication is frequently observed in patients with chronic schizophrenia. We herein present a 49-year-old man who developed schizophrenia at the age of 23 and had been admitted to the closed ward of our hospital for 7 years. He was found by a round nurse standing at the bedside, covering both ears with his hands and making groaning noises. He was disoriented and immediately after being returned to bed, a general tonic-clonic seizure occurred. Severe hyponatremia (Na 104 mEq/L) was noted and intravenous sodium correction was started. A few hours later, due to glossoptosis and massive vomiting, ventilation got worse to the point where he had to be put on a ventilator. On the following day, he developed aspiration pneumonia and antimicrobial treatment was started. In addition, a blood sample taken 36 hours later revealed an extensive elevation of creatine kinase (41,286 U/L), pointing to a possibility of rhabdomyolysis as a complication. Subsequently, the general condition gradually improved with antimicrobial therapy and sodium correction. He eventually recovered without any complications including central pontine myelinolysis. He had no history of polydipsia before this event but it was later found that esophageal stricture triggered complusive fluid intake, resulting in acute hyponatremia, seizure, aspiration pneumonia and rhabdomyolysis. A brief discussion will be provided on the issues surrounding hyponatremia, rhabdomyolysis and schizophrenia.

Central pontine myelinolysis: a rare finding in hyperosmolar hyperglycemia.

Central pontine myelinolysis (CPM) is a heterogeneous nervous system disease of pontine demyelination, usually caused by rapid correction of hyponatremia. In the present study, we report a unique case of a 46-year-old man with a hyperglycemic state complicated with CPM. MRI demonstrated a high signal on T2 and symmetric restricted diffusion in the pontine. In conclusion, the clinical case described confirmed that the hyperosmolar state inherent in hyperglycemia was a likely cause of CPM.

Delayed Presentation of Osmotic Demyelination Syndrome Treated With Plasmapheresis.

We present a unique case of a 42-year-old gentleman with alcohol use disorder who developed osmotic demyelination syndrome (ODS) despite appropriate hyponatremia correction. This patient initially presented with severe hyponatremia (Na 97 mEq/L) due to beer potomania, which was corrected gradually over eight days, resulting in no observed neurological deficits upon discharge. However, he was readmitted with respiratory failure from aspiration pneumonia, leading to endotracheal intubation. Laboratory findings revealed a sodium level of 134 mEq/L and serum osmolality (293 mOsm/kg). The patient had neurological exam findings of spontaneous eye opening with left gaze preference and decreased power ⅕ in all extremities. Following extubation, he experienced a relapse with evolving subacute central pontine myelinolysis and bulbar weakness necessitating reintubation. Subsequently, five sessions of plasmapheresis were conducted, resulting in stable clinical findings. Despite remaining non-verbal, the patient demonstrated gradual neurological motor improvement, progressing from 1/5 power in all extremities to 4/5 on the right side and 3/5 on the left side. He was discharged with ventilator support, tracheostomy, and PEG tube placement to a long-term care facility. This case underscores the importance of vigilant monitoring in high-risk individuals following hyponatremia treatment because ODS presentation can be delayed.

Osmotic demyelination syndrome caused by rapid correction of hyperammonemia and continuous hyperbilirubinemia: a case report and review of the literature.

Osmotic demyelination syndrome (ODS) is an acute demyelinating disorder characterized by the loss of myelin in the center of the basis pons, defined as central pontine myelinolysis (CPM), and demyelination in locations outside the pons, defined as extrapontine myelinolysis (EPM). ODS including CPM and EPM is mainly caused by rapid correction of hyponatremia. However, there are several reports of ODS in medical conditions such as malnutrition; alcoholism; liver transplantation; malignancy; sepsis; and electrolyte imbalance including hypernatremia, hypokalemia, hypophosphatemia, and chronic illness. ODS caused by rapid correction of hyperammonemia or continuous hyperbilirubinemia without sodium fluctuations has rarely been reported. Because ODS may be irreversible, prevention is crucial. Herein, we report a case of ODS secondary to rapid correction of hyperammonemia and continuous hyperbilirubinemia.

Central Pontine Myelinolysis and Hypokalemic Paralysis as Presenting Manifestations of Sjogren's Syndrome.

Neurological involvement in Sjogren's syndrome can have varied manifestations and can precede the classical sicca symptoms of Sjogren's syndrome. A 32-year-old woman presented with acute quadriparesis and dysarthria. She had severe hypokalemia, and an MRI of the brain showed a lesion in the central pons that was hyperintense on T2 and fluid-attenuated inversion recovery (FLAIR) sequences sparing the periphery, a trident appearance characteristic of central pontine myelinolysis (CPM). On further evaluation, she was found to have distal renal tubular acidosis (dRTA) due to primary Sjogren's syndrome. She was treated with steroids and other supportive measures, and she recovered completely in one month. We describe a mild form of CPM with classical MRI features in a patient with Sjogren's syndrome and hypokalemia due to dRTA.

A case of acute pulmonary complication due to botulinum toxin: Patient with central pontine myelinolysis developed acute respiratory distress syndrome after botulinum neurotoxin type A injection into spastic lower extremity muscles.

Chemodenervation with botulinum neurotoxin type A (BoNTA) is the preferred method for focal spasticity management among various treatment options. While BoNTA injection is considered safe, its widespread use and increasing evidence raise safety concerns. In this paper, we present a patient with central pontine myelinolysis, a rare disease, who developed acute respiratory distress syndrome on the third day after BoNTA application to the spastic gastrocnemius muscle group and required intubation in the intensive care unit due to this complication. To our knowledge, this is the first case reported in the literature to develop an acute pulmonary complication after BoNTA injection into spastic lower extremity muscles.

A Case of Alcohol Withdrawal-Induced Central and Extrapontine Myelinolysis.

A 40-year-old female with a history of chronic alcohol use disorder presented with an acute intractable left-sided headache for three days and progressively worsening unsteady gait requiring a wheelchair to ambulate. The patient had a history of chronic alcoholism since 2019 but reported abstinence since September 2021. One month after quitting alcohol, she experienced a sudden deterioration in bilateral extremity neuropathy, forgetfulness, difficulty writing, and severe mood swings, which continued to worsen until her presentation in July 2022. Laboratory tests, including complete blood count and electrolyte levels, were within normal ranges. A previous MRI performed during the investigation for alcoholic neuropathy a few months before she quit drinking showed no abnormalities. However, a subsequent MRI during work-up for the current acute symptoms revealed significant signal abnormalities involving the central pons, bilateral cerebral peduncles, and medullary pyramids, consistent with chronic central pontine myelinolysis (CPM) with extrapontine myelinolysis (EPM) extending into the peduncles. The patient received treatment with folate and multivitamins and was scheduled for outpatient follow-up with physical therapy for rehabilitation. This case highlights CPM as a consequence of alcohol withdrawal and emphasizes the importance of timely diagnosis and appropriate management in such patients.

Central pontine myelinolysis after liver transplant: A case report and an updated review.

Based on brain magnetic resonance imaging results, the second and third most frequent causes of central pontine myelinolysis (CPM) were liver transplant (LT) and cirrhosis, which together accounted for 13.7% and 12.5% of all diagnoses of CPM. Medical diseases including cirrhosis, LT, malnutrition, and alcoholism are well-known conditions associated with CPM in addition to hyponatremia. The prognosis is poor and depends on early diagnosis and treatment to achieve better outcomes. This is a case report of CPM occurring after a deceased donor LT in a patient presenting many risk factors such as chronic hyponatremia, previous episodes of encephalopathy, and tacrolimus immunosuppression. We briefly discuss the pathophysiology, risk factors, diagnosis, and treatment, highlighting the role and challenges of the anesthesiologist in managing this kind of patient and preventing the syndrome in the context of major surgery.

Rapid Correction of Hyponatremia With Isotonic Saline Leading to Central Pontine Myelinolysis.

Central pontine myelinolysis (CPM) is a part of the spectrum of osmotic demyelination syndrome (ODS), which is a rare demyelinating disorder due to the rapid correction of low serum sodium. It affects the neurons of the pons but may also involve other extra-pontine sites. The disease is characterized by a wide variety of clinical features ranging from dysarthria, dysphagia, bulbar palsy, quadriplegia, and behavioral and psychiatric disturbances. We present a case of a young female who developed CPM due to rapid sodium correction after vomiting. She presented with quadriplegia and locked-in syndrome. The diagnosis is on the basis of clinical and radiographic features. Magnetic resonance imaging (MRI) of the brain is superior to computed tomography (CT) in detecting changes. It shows hyperintensities on T2-weighted images that are classically known as the trident sign in the region of the pons. The patient was managed supportively, and the family was counseled regarding the poor prognosis of the disease. Unfortunately, she met a fatal fate due to a complication of CPM that is aspiration pneumonia. It is, therefore, imperative to create more awareness regarding the disease, and measures should be taken for its prevention that includes correction of low sodium levels not greater than 10 mmol/L/day.

Primary Sjögren's syndrome with renal tubular acidosis and central pontine myelinolysis: An unusual triad.

Primary Sjögren's syndrome (pSS) is a complex, multisystem autoimmune disorder. It is characterized by lymphocytic infiltration of the exocrine glands. In the setting of pSS, the presence of systemic disease is an important prognostic determinant, but involvement of the kidney is uncommon. The triad of pSS, distal renal tubular acidosis (dRTA), and central pontine myelinolysis (CPM) is rare and potentially fatal. A 42-year-old woman presented with dRTA, profound hypokalemia, and CPM characterized by progressive global quadriparesis, ophthalmoplegia, and encephalopathy. Sjögren's syndrome was diagnosed based on sicca symptoms, clinical features, and strongly positive anti-SSA/Ro and anti-SSB/La autoantibodies. The patient responded well to electrolyte replacement, acid-base correction, corticosteroids, and subsequent cyclophosphamide therapy. Early recognition and appropriate treatment resulted in good kidney and neurological outcomes in this case. This report highlights the need to consider the diagnosis of pSS in unexplained dRTA and CPM, as it has a favorable prognosis if recognized and managed timeously.

A rare presentation of central pontine myelinolysis secondary to hyperglycaemia.

BACKGROUND: Central pontine myelinolysis (CPM) is a rare demyelinating disorder caused by the loss of myelin in the center of the basis pontis. CPM typically occurs with rapid correction of severe chronic hyponatremia and subsequent disturbances in serum osmolality. Although hyperglycaemia is recognized as a pathogenetic factor in serum osmolality fluctuations, CPM is rarely seen in the context of diabetes. CASE PRESENTATION: A 66-year-old Chinese male presented with a history of gait imbalance, mild slurred speech and dysphagia for two weeks. MRI showed the mass lesions in the brainstem, and laboratory examinations showed high blood glucose and HbA1c, as well as increased serum osmolality. The patient was diagnosed with CPM secondary to hyperosmolar hyperglyceamia and received insulin treatment as well as supportive therapy. After six weeks of followup, the patient had fully recovered to a normal state. CONCLUSION: CPM is a potentially fatal neurological condition and can occur in uncontrolled diabetes mellitus. Early diagnosis and timely treatment are crucial for improving the prognosis.

Osmotic Demyelination After Rapid Correction of Hyperosmolar Hyperglycemia.

Osmotic demyelination syndrome (ODS) is seen due to an overt rise in serum osmolality, most often during rapid correction of chronic hyponatremia. We present the case of a 52-year-old patient who presented with polydipsia, polyuria, and elevated blood glucose with rapid correction of glucose levels under five hours and developed dysarthria, left-sided neglect, and unresponsiveness to light touch and pain in the left extremities on the second day of hospitalization. MRI revealed restricted diffusion in the central pons, extending into extrapontine areas suggestive of ODS. Our case highlights the importance of cautious correction of serum hyperglycemia and monitoring serum sodium levels in patients with a hyperosmolar hyperglycemic state (HHS).