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.

A 44-Year-Old Alcohol-Dependent Man Who Recovered from Central Pontine Myelinolysis with Supportive Physical Therapy.

BACKGROUND Central pontine myelinolysis (CPM) includes symmetric demyelination of the central pons. CPM is a rare neurological disorder that generally develops after rapid correction of hyponatremia in individuals having underlying conditions, such as malnutrition, alcoholism, and severe burns. It can cause severe long-term disabilities. However, there is currently no pharmacotherapy capable of promoting remyelination, a process crucial for recovery from CPM. We present the case of a patient with alcoholism and malnutrition-related CPM, which developed following rapid correction of hyponatremia but then improved remarkably with supportive physical therapy. CASE REPORT A 44-year-old alcoholic and malnourished man was admitted to an emergency hospital for disorientation due to overdrinking, but later developed bulbar palsy after hyponatremia was unexpectedly, but rapidly, corrected. Axial scans of the diffusion-weighted brain MRI revealed a characteristic lesion known as a piglet sign in the central pons. Based on his underlying conditions, present episode of sodium correction, and MRI finding, the patient was diagnosed as having CPM, which progressively worsened, resulting in locked-in syndrome after 12 days. The patient was then transferred to a long-term care unit and received simple motion exercise daily, but no specific medication. His symptoms gradually improved, achieving discontinuation of tube feeding on day 21, independent walking on day 110, and discharge after 6 months. CONCLUSIONS This report highlights the importance of physical therapy, the potential of which is often underestimated despite its broad benefits for human health, as a readily applicable intervention for patients with CPM. Further understanding of mechanisms underlying exercise-induced myelination should contribute to establishing novel therapies for a wide spectrum of brain disorders.

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.

Central pontine myelinolysis secondary to rapid correction of hyponatremia historical perspective with Doctor Robert Laureno.

OBJECTIVES: Central pontine myelinolysis (CPM) is a neurological disorder characterized by damage to the myelin and oligodendrocytes in the pons. This review focuses on the history of CPM and the discovery of its association with the treatment of hyponatremia. METHODS: The author reviewed original publications regarding CPM, hyponatremia, and the treatment of hyponatremia. The author interviewed Dr. Robert Laureno who was a pioneer in CPM research with his animal work in dogs. RESULTS: Animal models demonstrated the role of the rapid correction of hyponatremia as causative of pontine and extrapontine myelinolytic lesions. Nevertheless, the importance of the speed of correction was widely denied. There were years of debates and only slow changes in expert guidelines. CONCLUSION: CPM occurs as a consequence of a rapid rise in serum sodium in individuals with chronic hyponatremia. It is recommended to increase plasma sodium concentration by no more than 8 to 10 mmol/L per 24 h in chronic hyponatremia.

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.

Follow-Up of Brain Single-Photon Emission Computed Tomography (SPECT) and Magnetic Resonance Imaging (MRI) in a Case of Seizure Caused by Osmotic Demyelination Syndrome.

BACKGROUND Osmotic demyelination syndrome (ODS) is an uncommon neurological disorder. Until the mid-1980s, the mortality rate was 90-100%, but more than half of patients now have a good prognosis. Early suspicion of ODS is important. However, radiologic findings of ODS are variable and scintigraphy findings have not been reported. CASE REPORT A 38-year-old man with alcohol abuse history was admitted due to electrolyte imbalance. On the 10th day of his hospital stay, he had a generalized tonic-clonic seizure. Brain perfusion SPECT showed asymmetrically hyperperfused and hypoperfused lesions. Brain MRI revealed diffuse T2 hyperintensity with mild diffusion restriction in the pons and hyperperfused lesions on brain SPECT. He was treated based on the diagnosis of hyponatremia and osmotic demyelination. After treatment, the asymmetric hyperperfusion was decreased. MRI showed that the cortical hyperintensity had resolved, with encephalomalacic change shown in the pons. CONCLUSIONS To the best of our knowledge, this is the first report showing changes in brain perfusion SPECT and MRI in an ODS patient with a seizure. This case report may be helpful to neurologists, radiologists, and nuclear physicians.

Central Pontine Myelinosis and Osmotic Demyelination Syndrome.

BACKGROUND: Osmotic demyelination syndrome (ODS), which embraces central pontine myelinolysis (CPM) and extrapontine myelinosis (EPM), is often underdiagnosed in clinical practice, but can be fatal. In this article, we review the etiology, patho- physiology, clinical features, diagnosis, treatment, and prognosis of ODS. METHODS: Pertinent publications from the years 1959 to 2018 were retrieved by a selective search in PubMed. RESULTS: The most common cause of ODS is hyponatremia; particular groups of patients, e.g., liver transplant recipients, are also at risk of developing ODS. The pathophysiology of ODS consists of cerebral apoptosis and loss of myelin due to osmotic stress. Accordingly, brain areas that are rich in oligodendrocytes and myelin tend to be the most frequently affected. Patients with ODS often have a biphasic course, the first phase reflecting the underlying predisposing illness and the second phase reflecting ODS itself, with pontine dysfunction, impaired vigilance, and movement disorders, among other neurological abnormalities. The diagnostic modality of choice is magnetic resonance imaging (MRI) of the brain, which can also be used to detect oligosymptomatic ODS. The current mainstay of management is prevention; treatment strategies for manifest ODS are still experimental. The prognosis has improved as a result of MRI-based diagnosis, but ODS can still be fatal (33% to 55% of patients either die or remain permanently dependent on nursing care). CONCLUSION: ODS is a secondary neurological illness resulting from a foregoing primary disease. Though rare overall, it occurs with greater frequency in certain groups of patients. Clinicians of all specialties should therefore be familiar with the risk constellations, clinical presentation, and prevention of ODS. The treatment of ODS is still experimental at present, as no evidence-based treatment is yet available.

Osmotic Demyelination Syndrome in Children.

Osmotic demyelination syndrome is an acute demyelination process that usually occurs several days following an osmotic stress. This syndrome is rare in adults (0.4% to 0.56%) and even more uncommon in children. We performed a review of all reported pediatric osmotic demyelination syndrome patients from 1960 to 2018. Among all 106 cases, 49 presented with isolated central pontine myelinolysis, 30 with isolated extrapontine myelinolysis, and 27 with combined central pontine myelinolysis and extrapontine myelinolysis. There was no gender preponderance, and the highest prevalence was noted between the ages one and five years. Magnetic resonance imaging remains the diagnostic modality of choice, and diffusion tensor imaging is now increasingly used for prognostication in osmotic demyelination syndrome. Sixty percent of the children had a complete neurological recovery. Current management of osmotic demyelination syndrome in children consists of supportive medical care, steroids, and intravenous immunoglobulin. Our review of the literature supports the hypothesis that steroids and immunoglobulins are potentially helpful, although additional controlled studies are needed.

Central Pontine Myelinolysis After Living-Donor Liver Transplant: A Report of 2 Cases.

Here, we present 2 patients who developed central pontine myelinolysis after living-donor liver transplant. Both patients had abnormal sodium level before living-donor liver transplant. Patient 1 presented with severe hyponatremia on admission. After administration of 3% saline, her sodium level during the first 24 hours was kept at 100 mEq/L and then increased to 116 mEq/L during the next 24 hours. The level increased 5.8 mEq/L during the 4- to 5-hour transplant procedure. Patient 2 was admitted to the hospital with an unprovoked seizure. The serum sodium concentration was 111 mEq/L, which was treated with 3% saline infusion. Serum sodium concentration escalated to 118 mEq/L over an 8-hour period. Intraoperatively, both patients received large amounts of replacement fluids (0.9% normal saline and albumin), blood transfusion, and sodium bicarbonate during the anhepatic phase, all of which carry high sodium load. Variations in sodium levels changed rapidly in patient 1 during transplant surgery. After they underwent liver transplant, patient 1 had clear mental status and patient 2 demonstrated worsened mental status. On approximately day 14 and day 4 after liver transplant, magnetic resonance imaging showed diffuse abnormalities of the pons, resulting in diagnosis of central pontine myelinolysis. Although both patients survived, 1 remains in a vegetative state and the other continues to present with mild balance and swallowing abnormalities. To reduce the chance of inadvertent overcorrection in patients with hyponatremia, it is therefore important that sodium concentrations should be monitored frequently and fluids and electrolytes titrated carefully.

Osmotic Demyelination Syndrome Revisited: Review With Neuroimaging.

Osmotic demyelination syndrome (ODS) is a general term that has become commonplace in the practice of medicine, encompassing both central pontine myelinolysis and extrapontine myelinolysis. Historically ODS arises as a serious complication of rapid correction of hyponatremia, yet its manifestations seem to be influenced by a multifactorial process. Further understanding of this rare demyelinating disease has elucidated the significant role of other electrolyte disturbances and the presence of chronic comorbidities as disease risk factors. This review discusses the current research regarding the pathophysiology, clinical manifestations, neuroimaging features, patient management, and prognosis of osmotic demyelination syndrome. We hope that this review will further endorse and aid in the proper diagnosis of ODS and its suitable management through the understanding of clinical and imaging correlations and outcomes, and the comorbid factors that may predispose the development of ODS in certain patient populations.

Temporal evolution of the trident and piglet signs of osmotic demyelination syndrome.

Central pontine myelinolysis (CPM) is a potentially-devastating complication of rapid osmolar shifts, classically attributed to overlyaggressive correction of chronic hyponatremia. Magnetic resonance imaging (MRI) allowed earlier diagnosis of CPM, but most importantly, it has revealed that the odds of good functional recovery are surprisingly high. A trident shaped pontine lesion is a typical finding in CPM (the trident sign). The "piglet sign" is a much less well-known radiologic finding in CPM. Due to the rarity of CPM, very little has been published on the evolution of these MRI findings. We present a case of CPM in an alcoholic young man, and describe the temporal evolution of both the trident and piglet signs on MRI in CPM.

Central pontine myelinolysis during pregnancy: Pathogenesis, diagnosis and management.

Central pontine myelinolysis (CPM) is a rare condition usually caused by rapid sodium correction in hyponatraemia after a severe neurological syndrome. Only few cases have been reported during pregnancy, most of which were reported in patients with hyperemesis. We describe the successful management of the first case of twin pregnancy in a patient who presented with CPM after treatment for premature labour and then review the literature on CPM in pregnancy (aetiology, diagnosis and management). Our patient required emergency delivery to achieve electrolyte and fluid balance. At six months, the twins remained asymptomatic and the mother had minor sequelae. The aetiology is not clear, and there is no evidence regarding the optimal treatment or prognosis of CPM. In our patient, desmopressin-contaminated atosiban showed a certain probability in the Karch-Lasagne algorithm of a causality relationship between hyponatraemia and the drug. To our knowledge, this is the first case of myelinolysis reported in a twin pregnancy possibly related to desmopressin-contaminated atosiban.

Is Central Pontine Myelinolysis Reversible?

Central pontine myelinolysis (CPM) is a rare phenomenon that causes significant morbidity and mortality. Active therapeutic interventions for CPM can have a positive impact on recovery and overall prognosis. This case represents a 34-year-old white man with a chronic history of alcohol abuse who had Parkinsonian symptoms 13 days after rapid correction of his serum sodium in the hospital. Similarly to prior CPM case reports, this patient significantly improved following reinduction of hyponatremia, methylprednisolone, and/or plasmapharesis. This report demonstrates that CPM is potentially reversible when quickly recognized and therapeutic interventions are initiated rapidly.

Extrapontine myelinolysis manifested selectively by acute severe parkinsonian syndrome. Case Report.

OBJECTIVES: Osmotic demyelination syndrome (ODMS) is a rare and serious neurologic disorder with acute myelin disintegration, usually in the pontine area (central pontine myelinolysis) and to a lesser extent, even in other areas of the central nervous system (extrapontine myelinolysis). The main underlying mechanism is the change of serum osmolality with quick correction of low mineral levels, mainly hyponatraemia. Clinical manifestation is various and depends on the localization. DESIGN: We describe an acute isolated extrapontine myelinolysis causing acute onset of parkinsonism in a 61-year-old man who developed quickly progressing parkinsonian syndrome after the rapid correction of hyponatraemia. RESULTS: Brain MRI revealed lesions only in the striatum, sparing the globus pallidus. Substitution therapy with high doses of levodopa significantly improved his clinical condition. CONCLUSION: Extrapontine myelinolysis with isolated affection of basal ganglia is extremely rare. In such case, clinical manifestation of acute severe parkinsonism could be successfully treated by high dose of levodopa.

[Case of neuromyelitis optica spectrum disorder associated with central pontine and extrapontine myelinolysis preceded by syndrome of inappropriate antidiuretic hormone secretion].

A 36-year-old woman complained of general malaise. She presented with hyponatremia and plasma osmotic pressure was lower than urinary osmotic pressure. In addition, serum antidiuretic hormone level was higher than the measurement sensitivity. She was diagnosed with the syndrome of inappropriate antidiuretic hormone secretion (SIADH). She fell into a coma despite correction of serum sodium level. Brain magnetic resonance imaging (MRI) revealed high signal intensities in the cerebral cortex, striatum, thalamus, hypothalamus, midbrain, and pons in fluid-attenuated inversion recovery images. Spinal MRI revealed a longitudinally extending lesion in the cervical cord. Serum sample was positive for anti-aquaporin-4 antibody, supporting the diagnosis of neuromyelitis optica spectrum disorder (NMOSD) combined with central pontine and extrapontine myelinolysis. In patients with NMOSD, the immune reaction can gradually cause destructive changes of the hypothalamus and lead to unstable ADH secretion in the absence of immunomodulatory treatment.