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.

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.

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.

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.

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 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.

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.

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.

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.

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.

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).

Central Pontine Myelinolysis as a Late Complication After Hyponatremia and COVID-19 Infection.

Osmotic demyelination syndrome (ODS) is a rare but serious condition that is hypothesized to be a result of rapid correction of hyponatremia, with a catastrophic prognosis. The foci of demyelination may occur in either the pontine area or within the white matter of the cerebral hemispheres, which denotes a specific clinical presentation. We present the case of a post-COVID-19 patient who was diagnosed with ODS with typical clinical and radiological characteristics of both forms: central pontine myelinolysis and extrapontine myelinolysis. The clinical assessment of ODS encompasses a variety of differential diagnoses, including stroke, neuroinfection, neoplasia, and other demyelinating diseases. A specific characteristic of ODS is the delayed clinical manifestation after the hyponatremic state. Furthermore, it is noteworthy that there might be discrepancies between the clinical manifestations and the neuroimaging findings. The association between COVID-19 and ODS is unclear at the moment, although it can possibly be explained by the viral infection through multiple mechanisms such as renal dysfunction, diarrhea, or vomiting. ODS should be considered in cases of hyponatremia and neurological deterioration during the course of COVID-19 infection. Despite the fact that early detection and treatment of this syndrome can reduce the risk of short-term mortality and long-term disability, they do not guarantee complete recovery.

Central pontine myelinolysis and locked-IN syndrome associated with tacrolimus after pediatric heart transplantation.

BACKGROUND: Locked-in syndrome represents the most severe form of central pontine myelinolysis and varies in presentation from asymptomatic to fully developed locked-in-syndrome characterized by the combination of quadriplegia, loss of the ability to communicate except through the use of the eyes, and an inability to follow commands. METHODS: We report a 10-year-old boy who developed a severe case of locked-in syndrome after heart transplantation. RESULTS: Patient had a spontaneous recovery, treated with supportive treatment and the improvement was detected with cessation of calcineurin inhibitor therapy by substituting with an mTOR inhibitor (sirolimus). No cases of locked-in syndrome post-heart transplant in pediatrics cases have been documented in the literature. CONCLUSION: Physicians should recognize a rapid progression of central pontine myelinolysis and locked-in syndrome in the context of heart transplant and although several factors likely contributed to this outcome, adjustment of immunosuppression including by substituting tacrolimus with sirolimus could be effective.

A novel association of osmotic demyelination in Sjögren's syndrome prompts revisiting role of aquaporins in CNS demyelinating diseases: A literature review.

BACKGROUND: Primary Sjögren's syndrome (SS) is a chronic systemic autoimmune disease with varied neurological manifestations. SS is associated with anti-aquaporin-4 antibody (AQP4-IgG)-positive neuromyelitis optica spectrum disorder (NMOSD), a demyelinating autoimmune disorder of the central nervous system (CNS). Intriguingly, there are reports of osmotic demyelinating syndrome (ODS), a supposedly non-inflammatory disorder, in the context of SS and renal tubular acidosis (RTA), both of which are not yet established risk factors for ODS. METHODS: A literature search was undertaken to identify case reports of ODS in patients with SS. Details of the clinical and laboratory features of these patients were compiled. Additionally, we searched for NMOSD in patients with SS. We looked for co-existing RTA in patients with SS-ODS as well as SS-NMOSD. We also screened for reports of ODS in RTA without underlying SS. RESULTS & DISCUSSION: We identified 15 patients (all women, median age 40 years) with ODS in SS, and all of these patients had comorbid RTA. There were only three reported cases of ODS in RTA without underlying SS. We identified a total of 67 patients with SS-NMOSD, of whom only 3 (4.5%) had RTA. Hence, unlike NMOSD, the development of ODS in SS requires a prolonged osmotic or electrolyte abnormality caused by the comorbid RTA. The 15 patients with ODS and SS -RTA, showed heterogeneous clinical manifestations and outcomes. The most common symptom was quadriparesis, seen in 14 of the 15 patients. Eleven of the 15 patients had one of the following features, either alone or in combination: worsening of the sensorium, extensor plantar response, dysphagia/dysarthria, and facial palsy. The latter four manifestations were present at the onset in 7 patients and later in the course of the illness in the remaining 4 patients. Ocular palsy was seen in only four of the 15 patients and was a late manifestation. One patient who had extensive long-segment myelitis and subsequent ODS died, but most patients recovered without significant sequelae. None had hyponatremia, while all patients had hypokalemia and/or hypernatremia. Hypokalemia causing nephrogenic diabetes insipidus (NDI) followed by rapid rise in sodium and the resultant osmotic stress could potentially explain the occurrence of ODS in SS-RTA. Aquaporin (AQP) in astrocytes is implicated in ODS, and renal AQP is downregulated in NDI. Antibodies against AQPs are present in some patients with SS. Defective AQP is therefore a common link underlying all the connected diseases, namely SS, NDI, and ODS, raising the possibility of immune-mediated AQP dysfunction in the pathogenesis. CONCLUSION: The hitherto unreported association between SS-RTA and ODS may implicate SS and/or RTA in the development of ODS. In the setting of SS-RTA, ODS must be suspected when a patient with flaccid quadriparesis does not respond to the correction of potassium or develops additional neurological features along with a rise in sodium. Defective functions of AQPs may be a possible mechanism linking demyelinating CNS lesions, SS, and RTA. Studies evaluating AQP functions and serum antibodies against AQPs in these conditions are warranted.

A multidisciplinary advanced approach in central pontine myelinolysis recovery: considerations about a case report.

BACKGROUND AND PURPOSE: Central pontine myelinolysis (CPM) is a solitary, symmetric, demyelination in the central pons. This case study aimed to report the effects of an intensive robotic gait training with Lokomat-Pro on mobility and quality of life in a case of CPM. CASE DESCRIPTION AND INTERVENTION: A 33-year-old female patient with tetraparesis and gait disturbance due to CPM was hospitalized to undergo intensive rehabilitation training for about 2 months. Daily session of Lokomat-Pro and psychotherapy by telemedicine were performed, besides nursing care and occupational and physical therapy. Motor evaluation and quality of life were assessed by using standardized scales. OUTCOMES: The multidisciplinary therapy led to significant improvements both in functional motor outcomes (as per 10-Meter Walk Test, Berg Balance and Tinetti scale) and quality of life. DISCUSSION: Innovation technology, including robotics and telemedicine, may be a valuable tool to improve functional outcomes in patients with severe motor impairment due to chronic CPM.IMPLICATIONS FOR REHABILITATIONA multidisciplinary approach involving robotics plus virtual reality is mandatory to reduce medical and bedridden complications in patients affected by CPM.

Osmotic demyelination syndrome: novel risk factors and proposed pathophysiology.

BACKGROUND: Osmotic demyelination syndrome (ODS) is non-inflammatory demyelination in response to an osmotic challenge. It can be pontine or extrapontine in presentation. AIMS: To retrospectively review cases involving ODS and define the spectrum of causes, risk factors, clinical and radiological presentations, and functional outcomes. RESULTS: The study utilised data from 15 patients with a mean age of 53.6 years. Malnutrition (9; 60%) and chronic alcoholism (10; 66.7%) were the most common associated disorders. Two (13.3%) patients had severe hyponatraemia (<120 mmol/L). The average highest single-day change was 5.1 mmol/L. Radiologically, 14 (93.3%) had pontine and 6 (40%) had extra-pontine lesions. Hypokalaemia (14; 93.3%) and hypophosphataemia (9; 60%) were commonly associated. Common clinical manifestations include altered consciousness/encephalopathy (9; 60%), dysphagia (4; 26.7%) and limb weakness (4; 26.7%). At 3 months, two (14.3%) had died and six (40%) were functionally independent (modified Rankin scale 0-2). CONCLUSION: We found that ODS occurred despite appropriate correction rates of hyponatraemia. Factors such as malnutrition, chronic alcoholism, hypokalaemia and hypophosphataemia are thought to play a role in its pathogenesis. Approximately half of the patients survived and became functionally independent.