Therapy of central pontine myelinolysis following livingdonor liver transplantation:Report of three cases

We analyzed the clinical manifestations and experiences of diagnosing and treating central pontine myelinolysis following living donor liver transplantation. The clinical data of three patients with central pontine myelinolysis following living donor liver transplantation from January 2005 to November 2007 were retrospectively analyzed at the West China Hospital, Sichuan University, China. The three patients developed hyponatremia prior to surgery. Case 1 suffered locked-in syndrome following surgery, and received a large dose of gamma globulin, and subsequently recovered. Case 2 was in a coma for three days, and received hyperbaric chamber treatment. This patient remained in a mild coma for six months following surgery. Case 3 developed consciousness disturbances, gradually went into a coma following surgery, and died due to pulmonary infection. Central pontine myelinolysis is a severe complication in patients following living donor liver transplantation. Large-dose gamma globulin treatment, as well as hyperbaric oxygen, might be effective therapeutic methods.

Extrapontine myelinolysis caused by rapid correction of pituitrin-induced severe hyponatremia: A case report

BACKGROUND Severe hyponatremia is considered a rare complication of pituitrin,which is widely used for the treatment of pulmonary hemorrhage.However,the management of pituitrin-associated hyponatremia can be challenging because a rapid correction of hyponatremia may cause the development of osmotic demyelination syndrome,resulting in life-threatening neurological injuries.CASE SUMMARY A 20-year-old Chinese man with massive hemoptysis developed symptomatic hyponatremia(116 mmol/L)after therapy by a continuous intravenous drip of pituitrin.To normalize his serum sodium,a hypertonic saline infusion was applied for 3 d,and the pituitrin administration was stopped concurrently.Then,an overly rapid increase in serum sodium level(18 mmol/L in 24 h)was detected after treatment.One day later,the patient experienced a sudden onset of generalized tonic-clonic seizures,as well as subsequent dysarthria and dystonia.Magnetic resonance imaging revealed increased signal intensity in the bilateral symmetric basal ganglia on the T2-weighted images,compatible with a diagnosis of extrapontine myelinolysis.The patient received an intravenous administration of high-dose corticosteroids,rehabilitation,and neurotrophic therapy.Finally,his clinical abnormalities were vastly improved,and he was discharged with few residual symptoms.CONCLUSION Physicians should be fully aware that pituitrin can cause profound hyponatremia and its correction must be performed at a controlled rate to prevent the development of osmotic demyelination syndrome.

Central pontine myelinolysis mimicking glioma in diabetes:A case report

BACKGROUND Central pontine myelinolysis(CPM)usually occurs during rapid correction of serum osmolality,typically with brainstem lesions presenting uniform signals following enhancement on magnetic resonance imaging(MRI).We report a case of CPM caused by diabetes,which was characterized by glioma-like imaging features and the patient responded well to corticosteroids.CASE SUMMARY A 49-year-old man with type 2 diabetes was admitted due to numbness and weakness for 6 mo with progressive aggravation for 2 wk.His complete blood count,serum electrolytes,renal and liver function parameters were within the normal range.MRI showed mass lesions in the brainstem,with unusually inhomogeneous signal intensity after contrast-enhanced scans.His symptoms worsened after hypoglycemic therapy.Due to his clinical history and examination results,CPM was considered the most likely diagnosis.Treatment with corticosteroids was administered with a methylprednisolone pulse in the acute phase followed by dose tapering.During the 8-mo follow-up period,his clinical symptoms and imaging features significantly improved.CONCLUSION Diabetes could rarely be accompanied by CPM,and patients who experience this neurological complication could benefit from corticosteroid treatment.Clinicians should recognize the special relationship between diabetes and CPM,and improve awareness of early identification and appropriate treatment.

Structural neuroimaging and neuropsychological correlates of a single case of focal central pontine myelinolysis: Intact memory function with decreased intellectual and motor functions

We report on the case of middle-aged right-handed woman with central pontine myelinolysis (CPM) revealed by high resolution structural T2-weighted FLAIR MRI imaging. There was a general flattening of Wechsler Adult Intelligence Scale—Fourth Edition subtest scores which were 1 standard deviation below expected values. In contrast Wechsler Memory Scale—Fourth Edition visual and auditory memory scores remained within the normal range. Verbal working memory appeared mildly impaired while nonverbal working memory was not. Scores on the Advanced Clinical Solution’s Social Perception battery were all in the normal range as were academic skills measured by the Wide Range Achievement Test—Fourth Edition. Performance was impaired on the Delis-Kaplan Executive Function System’s counterpart of the Trail-Making Test: Part B. Similarly, on the Draw-A-Person Test there was a discrepancy in that our patient’s standard score was 76 compared to her estimated premorbid FSIQ in the average range. She also displayed bilateral motor coordination slowing on the Finger Tapping task collectively suggesting damage to pontine motor tracts. The Minnesota Multiphasic Personality Inventory—Second Edition—Restructured Form profile was consistent with a diagnosis of severe anxiety and depression perhaps due to damage to serotoninergic neural tracts originating within the central pons. Finally, the patient displayed severe sleep disturbances and other signs of reticular activating formation injury. CPM may constitute a unique means of studying reversible subcortical lesions in the central pons in otherwise healthy subjects with benign illness. To our knowledge this is among the first patients with CPM without the usual risk factors for the disorder and who was otherwise healthy. Knowledge of the etiology and neuropsychology of such patients might aid in understanding the interaction of the fronto-ponto-cerebellar tracts in executive functions and motor programming.

Rehabilitation of anterior pituitary dysfunction combined with extrapontine myelinolysis: A case report

BACKGROUND Extramedullary myelinolysis is a rare demyelinating disease, often caused by rapid increases in serum sodium concentration in patients with hyponatremia.Clinical manifestations are neuropsychiatric symptoms, limb weakness, and dysarthria. Because of its poor prognosis and high disability rate, it poses a huge burden on the global economy, societies, and families. This article reports rehabilitation in a patient with pituitary dysfunction combined with extramedullary myelinolysis.CASE SUMMARY A 27-year-old Chinese man developed anorexia, vomiting, and limb weakness and was diagnosed with pituitary insufficiency. He had low serum sodium, slow movement, muscle weakness, and muscle tone abnormalities after sodium supplementation, involuntary limb shaking, ataxia, and dysarthria. According to the symptoms and signs and imaging reports, he was diagnosed with extramedullary myelinolysis. After treatment with hormone therapy and neurotrophic drugs, motor and speech function did not improve, so he was treated in the rehabilitation department for 4 wk. The patient's physical status was improved substantially during his stay at the rehabilitation department.CONCLUSION Patients with extramedullary myelinolysis who actively participate in rehabilitation intervention can significantly improve their activities of daily living.

Centropontine Myelinolysis after Conservative Correction of Hyponatremia: A Case Report and Review of Contributing Factors

Introduction: Centropontine myelinolysis (CPM) is a rare pathology, a delayed neurological complication corresponding to massive demyelination of the protrusion. Its exact pathogenesis is poorly understood. Rapid correction of sodium hyponatremia has been implicated as a potent causative factor. We report a case of CPM despite a priori conservative correction of hyponatremia with a favorable course in a 61-year-old alcoholic-smoker diabetic. Case Presentation: A 61-year-old man with chronic alcoholism presented to the emergency department (D0) with physical asthenia and anorexia. He was treated for severe hyponatremia at 104 mmol/L by careful rehydration with saline before being transferred to a nephrological hospital. Magnetic resonance imaging (MRI) performed at D14 for locked-in syndrome showed osmotic demyelination syndrome (Figure 1). The evolution was favorable after 3 months of rehabilitation marked by a progressive and clear improvement of clinical signs. Conclusion: This observation suggests an evaluation of the benefit/risk ratio of the short-term prognosis of profound hyponatremia with that of the metabolic stress induced by a still too rapid correction. Particular attention should be paid to diabetic patients in the context of chronic alcoholism or nutritional deficiencies.

Case Report:Pure word deafness associated with extrapontine myelinolysis

Extrapontine myelinolysis and pure word deafness are very uncommon disorders.Here,we report a case of a 19-year-old woman who suffered from osmotic demyelination syndrome with coincidence of typical pure word deafness.As a consequence of rapid correction of hyponatremia,the patient demonstrated an initial onset of cortical deafness,and then progressed to generalized auditory agnosia,which eventually developed into confined verbal auditory agnosia(pure word deafness) .Bilateral extrapontine myelinolysis was confirmed using brain magnetic resonance imaging.This case suggests that verbal and nonverbal stimuli may involve separate thalamocortical pathways.

Caudal and Lentiform nuclei Myelinolysis following Endoscopical surgery for pediatric Craniopharyngioma: two cases report and literature review

Background: The extrapontine myelinolysis (EPM) of osmotic demyelination syndrome (ODS) is a rare and dangerous disorder of the central nervous system. The mechanism is not fully understood and the treatment is controversial. There are few reports of EPM occurred after endonasal endoscopic resection of pediatric craniopharyngioma. Case presentation: We report two unusual cases of such a rare condition. Both are procedured uneventful endonasal endoscopical craniopharyngioma resection, after operation, the patients suffered serious diabetic insipidus with remarkable fluctuation of serum sodium though we applied pitutrin continual management to maintain everyday normal urine and correct serum sodium disorder. MR images occured caudal and lentiform nuclei myelinolysis following these serious conditions. Conclusions: In this study relevant literatures are reviewed in order to further understand this complication and contribute to the prevention and treatment of EPM caused by inappropriate correction of serum sodium disorder.

Reversible lesions in the brain parenchyma in Wilson's disease confirmed by magnetic resonance imaging:earlier administration of chelating therapy can reduce the damage to the brain

The aim of this study was to evaluate the resolution of brain lesions in patients with Wilson’s disease during the long-term chelating therapy using magnetic resonance imaging and a possible signiifcance of the time latency between the initial symptoms of the disease and the introduction of this therapy. Initial magnetic resonance examination was performed in 37 patients with proven neurological form of Wilson’s disease with cerebellar, parkinsonian and dystonic presentation. Magnetic resonance reexamination was done 5.7 ± 1.3 years later in 14 patients. Patients were divided into： group A, where chelating therapy was initiated 〈 24 months from the ifrst symp-toms and group B, where the therapy started≥ 24 months after the initial symptoms. Symmetry of the lesions was seen in 100% of patients. There was a signiifcant difference between groups A and B regarding complete resolution of brain stem and putaminal lesions （P= 0.005 andP=0.024, respectively）. If the correct diagnosis and adequate treatment are not established less than 24 months after onset of the symptoms, irreversible lesions in the brain parenchyma could be ex-pected. Signal abnormalities on magnetic resonance imaging might therefore, at least in the early stages, represent reversible myelinolisis or cytotoxic edema associated with copper toxicity.

Desmopression is an effective adjunct treatment for reversing excessive hyponatremia overcorrection

We report a case of a 50-year-old malnourished African American male with hiccups, nausea and vomiting who was brought to the Emergency Department after repeated seizures at home. Laboratory evaluations revealed sodium(Na+) 107 mmol/L, unmeasurably low potassium, chloride < 60 mmol/L, bicarbonate of 38 mmol/L and serum osmolality 217 m Osm/kg. Seizures were controlled with 3% saline Ⅳ. Once nausea was controlled with iv antiemetics, he developed large volume free water diuresis with 6 L of dilute urine in 8 h(urine osmolality 40-60 m Osm/kg) and serum sodium rapidly rose to 126 mmol/L in 12 h. Both intravenous desmopressin and 5% dextrose in water was given to achieve a concentrated urine and to temporarily reverse theacute rise of sodium, respectively. Serum Na+ was gradually re-corrected in 2-3 mmol/L daily increments from 118 mmol/L until 130 mmol/L. Hypokalemia was slowly corrected with resultant auto-correction of metabolic alkalosis. The patient discharged home with no neurologic sequaele on the 11 th hospital day. In euvolemic hyponatremic patient, controlling nausea may contribute to unpredictable free water diuresis. The addition of an antidiuretic hormone analog, such as desmopressin can limit urine output and prevent an unpredictable rise of the serum sodium.