Locked-in syndrome after central pontine myelinolysis, an outstanding outcome of two patients.

OBJECTIVE: Central pontine myelinolysis (CPM) is a rare demyelinating disease that affects the pons and which can cause extreme disabilities such as locked-in syndrome (LIS) in the initial phase. The aim of the study was to describe the evolution over a 12-month period of two patients with CPM causing an initial LIS. METHOD: We retrospectively report the unexpected clinical outcome of these two patients in relation with the anatomical damages documented by brain MRI, associated with diffusion tensor imaging and reconstruction of corticospinal tracts in tractography. The following clinical parameters systematically assessed at 3, 6, 9, and 12 months: muscle testing on 12 key muscles (Medical Research Council), prehension metrics (box and block test and purdue pegboard), and independence for acts of daily living (functional independence measure). RESULTS: Both patients showed a progressive recovery beginning between 2 and 3 months after the onset of symptoms, leading to almost complete autonomy at 12 months (FIM > 110), with motor strength greater than 4/5 in all joint segments (MRC > 50/60). On brain MRI with tractography, CST appeared partially preserved at pons level. INTERPRETATION: The possibility of a near-complete functional recovery at 12 months is important to consider given the ethical issues at stake and the discussions about limiting care that may take place initially. It seems to be the consequence of reversible myelin damage combined with partially preserved neurons. Development of collateral pathways or resolution of conduction block may explain this recovery. MRI comprising DTI and tractography could play a key role in the prognosis of motor recovery.

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.

Reversible Severe Parkinsonism Caused by Extra-Pontine Myelinolysis in a patient with Primary Adrenal Failure.

PURPOSE: Extrapontine myelinolysis (EPM) is a highly uncommon, life-threatening disease, particularly in individuals who initially appear with severe clinical symptoms. Here, we describe a case of EPM caused by the rapid correction of hyponatremia that had severe clinical signs at first but parkinsonism symptoms were fully improved after treatment. CASE REPORT: A 46-year-old female patient was admitted to the hospital due to impaired consciousness. Her medical history reveals that she has PAI, or primary adrenal insufficiency. Initial laboratory measurements showed that the serum's sodium (Na) concentration was 104 mEq/L, chloride (Cl) content was 70 mmol/L, potassium (K) content was 4.95 mEq/L, glucose was 42 mg/dL, hydrogen potential (Ph) was 7.12, and bicarbonate (HCO3) concentration was 10 mmol/l. The adrenocorticotropic hormone (ACTH) level was 21 mg/ml, while the cortisol level was 1.2ug/dl. Her mental state was unclear, she had sluggish hypophonic speech, generalized akinesia/rigidity in both upper and lower extremities, trouble swallowing solid and liquid meals, and sialorrhea were all discovered after the Na level was corrected. Hyperintense lesions were visible in the bilateral putamen and caudate nuclei of the Magnetic Resonance Imaging (MRI) T2 and flair-weighted scans, which indicate EPM. EPM was treated with corticosteroids and dopamine agonists, and she was eventually released after complete recovery. CONCLUSION: Even if there are severe clinical symptoms at first, prompt diagnosis and treatment, such as dopaminergic, corticosteroid, and palliative therapy, can save a patient's life.

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.

FDG PET/CT Aiding in Early Diagnosis of Central Pontine Myelinolysis.

ABSTRACT: We report a 70-year-old man with central pontine myelinolysis who presented in the emergency department with recent onset altered sensorium and tremors (Glasgow Coma Scale score, 13). Laboratory findings revealed hyponatremia and hypokalemia. MRI brain was unremarkable. Subsequently, patient was referred for FDG PET/CT to rule out malignancy or paraneoplastic syndrome. FDG PET revealed focal radiotracer uptake in the pons without any underlying CT abnormality consistent with central pontine myelinolysis, thus aiding in early diagnosis where conventional imaging modality was unremarkable.

Neuroleptic malignant syndrome in a case of extra-pontine myelinolysis: On the horns of dilemma.

Osmotic demyelination syndrome (ODS) and neuroleptic malignant syndrome (NMS) lead to severe neurological sequalae. Though currently thought to be different syndromes, literature suggests a relation between the two. We present the case of a 45-year-old male who was found to have chronic severe hyponatremia and underwent rapid correction of sodium and developed parkinsonism features. Magnetic resonance imaging (MRI) confirmed extrapontine myelinolysis (a type of ODS). The patient received haloperidol for agitated behavior and developed new features of rigidity, fever, tachycardia and elevated creatine phosphokinase (CPK) levels and thus neuroleptic malignant syndrome was suspected to overlap with ODS. We report this case highlighting the difficulty in differentiating the between ODS and NMS and their relationship.

Rare case of diffuse large B-cell lymphoma presenting with central pontine myelinolysis.

Central pontine myelinolysis (CPM) is commonly associated with osmotic stress and rapid correction of hyponatraemia. It has rarely been reported in conjunction with malignancies. We report a case where CPM was not only associated with a new diagnosis of diffuse large B-cell lymphoma but was also a key presenting feature.

Osmotic Demyelination Syndrome: Clinical, Neuroimaging Characteristics, and Outcomes in a Series of 18 Cases.

OBJECTIVE: To investigate the etiology, clinical as well as neuroimaging characteristics, and outcomes after proper treatment in a series of 18 patients with osmotic demyelination syndrome. METHODS: Medical records, including video records, of 18 patients with osmotic demyelination syndrome were retrospectively examined. Demographic and clinical information, imaging results, plans of management, and outcomes during the follow-up period were collected and analyzed. RESULTS: Eighteen patients, including 10 males and 8 females, were included in the present study. The mean age at diagnosis of CNS insult was 47.4 ± 13.3 years (ranged from 30 to 78 years). Etiologies included rapidly corrected hyponatremia (50%), alcoholism (27.8%), and others. Neurological manifestations included encephalopathy (61.1%), dysphonia (50%), extrapyramidal symptoms (38.9%), and seizures (22.2%). Neuroimaging results showed that 6 patients (33.3%) had central pontine myelinolysis, 5 (27.8%) had extrapontine myelinolysis, and 7 (38.9%) had both. After treatment, 12 patients showed improvement and the other 6 did not. Among these patients, those who showed symptoms of encephalopathy had a favorable outcome. The majority of those who presented with mental retardation, seizures, and no other symptoms recovered better than their counterparts who had other symptoms. Nine out of 11 patients with pseudobulbar paralysis and/or extrapyramidal symptoms showed improvement, but the other 2 did not show improvement. Five patients who did not improve after treatment during admission were followed up for 1-3 months with rehabilitation training recommended, and it was found that 3 showed significant improvement after training, and the other 2 did not respond to this training. CONCLUSIONS: Osmotic demyelination syndrome is a complex disease entity due to a variety of etiologies, manifesting with symptoms involving diverse systems of the brain. Early identification and removal/correction of conditions leading to osmotic demyelination syndrome are the key to prevent and/or manage this disease.

Incidentally Detected Hypermetabolism in Pons Suggestive of Pontine Myelinolysis on 18F-FDG PET/CT Scan.

ABSTRACT: Central pontine myelinolysis (CPM) is demyelinating condition of pons caused by osmotic stress due to rapid correction of hyponatremia. We present a case where CPM was an incidental finding on FDG PET/CT scan. To the best of our knowledge, only 3 case reports have been published till date describing CPM on 18F-FDG PET/CT scans.

Central pontine myelinolysis and the osmotic demyelination syndromes: an open and shut case?

Central pontine myelinolysis and extrapontine myelinolysis are collectively called the osmotic demyelination syndromes. Despite being described in 1959, there are several aspects of the disorder that remain an enigma. Animal models and neuroimaging techniques have allowed us to understand the condition better. From being a universally fatal disorder that was diagnosed post mortem, increased awareness, neuroimaging techniques and supportive care have enabled us to make the diagnosis ante-mortem. This has also led to a significant drop in associated mortality. The aim of this review is to highlight the clinical spectrum, neuroimaging findings, and recent developments.

Colchicine poisoning complicated by medulla oblongata myelinolysis: a case report.

Medulla oblongata myelinolysis is an extremely rare manifestation of extrapontine myelinolysis (EPM). Herein, we report a case of a 34-year-old man with a history of gout who presented repeated vomiting and diarrhea after ingesting 15 colchicine pills. A hyponatremia diagnosis was given and after an intensive treatment, his serum sodium level increased from 118 to 129 mmol/L within 24 hours. Brain magnetic resonance imaging (MRI) revealed a lesion in the medulla oblongata that appeared as a hypointense area in T1-weighted images and a hyperintense area in T2-weighted images. A diagnosis of medulla oblongata myelinolysis and colchicine poisoning was then given, and methylprednisolone therapy was initiated. Seventeen days later, the patient achieved a good outcome with methylprednisolone therapy. However, his medulla oblongata lesion remained detectable with MRI. Medulla oblongata myelinolysis is an extremely rare manifestation of EPM, and unique for being colchicine-induced. This case shows that colchicine poisoning can lead to hyponatremia, which in turn can induce myelinolysis if not treated correctly. As exemplified by our patient's case, desirable treatment outcomes are possible in such cases, although these outcomes may not be associated with a visible reduction of the brain lesions in MRI scans.