Asymptomatic pontine myelinolysis

A 43-year-old lady presented with bilateral foot drop due to alcohol-related peripheral neuropathy. There was no history of electrolyte disturbance or altered consciousness. Cranial nerve, bulbar and pyramidal symptoms and signs were absent. Nerve conduction studies confirmed the neuropathy. Inadvertently requested neuroimaging of brain demonstrated signal change typical of central pontine myelinolysis. Asymptomatic pontine myelinolysis occurs rarely in alcoholics in the absence of bulbar dysfunction. It is important for physicians to be aware of the clinical entity of asymptomatic pontine myelinolysis to avoid misinterpretation of abnormalities detected on cerebral imaging in alcoholic individuals.     

Brain-stem auditory evoked responses in diagnosis of central pontine myelinolysis

Summary Central pontine myelinolysis (CPM) developed in association with acute adrenocortical insufficiency during correction of severe hyponatraemia in a 58-year-old woman. Repeated CT scanning and NMR imaging were normal from the onset of the illness. Electroencephalography and brainstem auditory evoked responses showed abnormalities consistent with a brain-stem lesion, which resolved as the patient made a gradual but incomplete recovery. Our observations illustrate the value of electrophysiological monitoring in CPM and support the proposed association between this condition and the rapid correction of an electrolyte imbalance.     

脑桥中央及脑桥外髓鞘溶解症1例

脑桥中央及脑桥外髓鞘溶解症是常见的渗透性脱髓鞘疾病,近年来报道的病例逐渐增多,随着重症监护的发展、核磁共振的早期诊断,患者预后得到较大改善。现将解放军总医院神经内科重症监护室收治的1例脑桥中央及脑桥外髓鞘溶解症及诊治经验报道如下。     

Brachial diplegia in central pontine myelinolysis

Summary A patient developed weakness in the upper limbs, eventually causing brachial diplegia with only slight paresis of the legs after rapid correction of severe hyponatraemia. Pseudobulbar palsy, mental confusion and urinary incontinence were also present. CT scan showed a zone of lucency in the pons. Clinical recovery occurred and the zone of lucency had disappeared 12 months after the appearance of the neurological signs.     

Recovery after central pontine myelinolysis

Summary Central pontine myelinolysis (CPM) is known to be almost universally fatal. Most published cases are based on autopsy reports, whereas antemortem diagnosis is rare. We present a case in which the diagnosis of CPM was confirmed by computed tomography (CT); the patient had two symptoms not previously reported and made a remarkable recovery.

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Zusammenfassung Es ist bekannt, daß die zentrale pontine Myelinolyse praktisch immer tödlich endet. Die meisten veröffentlichten Fälle sind auf Grund der Autopsie diagnostiziert worden, während eine prämortale Diagnose selten ist. Der vorliegende Fall einer zentralen pontinen Myelinolyse wurde durch Computertomographie intra vitam bestätigt. Der Patient wies zwei früher nicht beschriebene Symptome auf und erholte sich in bemerkenswerter Weise.

     

Diffusion-weighted MR findings of central pontine and extrapontine myelinolysis

Diffusion-weighted MR (DWI) can detect changes in water diffusion associated with cellular dysfunction, which enables the differentiation of cytotoxic edema from vasogenic edema. In this study on DWI findings in central pontine (CPM) and extrapontine myelinolysis (EPM), DWI showed high signal intensities in the bilateral pons, midbrain, and genu of the corpus callosum. The corresponding apparent diffusion coefficient values were rather low. This suggests that cytotoxic edema does in fact exist in CPM and EPM and that DWI can be useful in the rapid diagnosis and prediction of the various types of edema occurring in active demyelinating diseases.     

Central pontine and extrapontine myelinolysis: a systematic review

The purpose was to perform a systematic review of studies on central pontine and extrapontine myelinolysis [forms of osmotic demyelination syndrome (ODS)] and define the spectrum of causes, risk factors, clinical and radiological presentations, and functional outcomes of this disorder. A thorough search of the literature was conducted using multiple databases (PubMed, Ovid Medline and Google) and bibliographies of key articles to identify all case series of adult patients with ODS published from 1959 to January 2013. Only series with five or more cases published in English were considered. Of the 2602 articles identified, 38 case series were included comprising a total of 541 patients who fulfilled our inclusion criteria. The most common predisposing factor was hyponatremia (78%) and the most common presentation was encephalopathy (39%). Favorable recovery occurred in 51.9% of patients and death in 24.8%. Liver transplant patients with ODS had a combined rate of death and disability of 77.4%, compared with 44.7% in those without liver transplantation (P < 0.001). ODS is found to have a good recovery in more than half of cases and its mortality has decreased with each passing decade. Favorable prognosis is possible in patients of ODS, even with severe neurological presentation. Further research is required to confirm the differences found in liver transplant recipients.     

Mild central pontine myelinolysis: a frequently undetected syndrome

Summary Over a period of 1 year we diagnosed central pontine myelinolysis (CPM) in five patients all of whom survived, two of them with complete functional recovery despite extensive lesions on cranial computerized tomography and magnetic resonance imaging.Diagnosis was based upon the combination of an acute brainstem dysfunction with typical neuroradiological features; a history of chronic alcoholism or a preceding hyponatremia may serve as a diagnostic hint.The spectrum of symptoms ranged from severe tetraplegia and cranial nerve palsies to latent signs of pyramidal tract lesions and discrete ocular motor abnormalities. In two patients pontine and extrapontine manifestations of demyelination were confirmed neuroradiologically; in one patient a solely extrapontine manifestation was present.Thus it is reasonable that: (1) the incidence of comparatively mild forms of CPM as well as extrapontine manifestations are more frequent than hitherto assumed, (2) the clinical outcome of the syndrome is better than expected from earlier fatal case reports and is quite independent of the extent of the lesion as it appears with brain imaging methods.     

Belly dancer's syndrome following central pontine and extrapontine myelinolysis.

We report on a woman with delayed-onset of belly dancer's syndrome 5 months after central pontine and extrapontine myelinolysis (CPM/EPM) and severe hyponatriemia. This case demonstrates that basal ganglia lesions in EPM can be the underlying pathoanatomic substrate for the rarely observed belly dancer's syndrome. The sequential appearance of extrapyramidal symptoms might reflect an ongoing but ineffective or deficient remyelination process. The presence of CPM/EPM should be considered in patients with involuntary dyskinesias of the abdominal wall.     

Central pontine myelinolysis associated with low potassium levels in alcoholism

Summary Two patients with chronic alcohol abuse and central pontine myelinolysis are described. One developed a Korsakoff syndrome 2 days before admission to our hospital and the other showed signs of a incipient delirium without Korsakoff syndrome. Diagnosis of incipient central pontine myelinolysis was based on acute brain-stem dysfunction, serum electrolyte disturbances, malnutrition with vitamin B₁ (thiamine), B₆ (pyridoxine) and B₁₂ (cyanocobalamine) deficiency in combination with typical neuroradiological findings. Hypokalaemia but no disturbance in serum sodium levels was found in both patients. After correction of hypokalaemia and vitamin deficiency the patients showed complete recovery of neurological and neuropsychological function. The findings are interpreted as suggesting that disturbances in serum potassium levels as well as rapid correction of hyponatraemia may be associated with pontine swelling and dysfunction which, if undetected, leads to central pontine myelinolysis.     

非钠离子快速纠正的脑桥中央髓鞘溶解的临床分析

脑桥中央髓鞘溶解（central pontine myelinolysis，CPM）是一种临床较为罕见的疾病，通常导致该病的原因是钠离子异常的快速纠正。该文报道了3例并非由钠离子快速纠正导致的CPM，其中2例为极高血糖并酮症酸中毒引起的，1例为钾离子过低引起的。经过治疗原发病和营养脑神经后，患者均好转出院。该文总结了这3个病例的临床特点，同时总结分析了国内外CPM相关的研究进展，希望能提高对于该病的认识。 [国际神经病学神经外科学杂志, 2023, 50(2): 49-52]     

Rare association of central pontine myelinolysis with infantile tremor syndrome

Central pontine myelinolysis (CPM) is an acute demyelination within the central basis pontis. Though exact mechanism is not known it is seen commonly with rapid correction of hyponatremia and also with pontine ischemia or infarction, demyelinating diseases, pontine neoplasm and different metabolic diseases. We report a rare association of CPM in a patient of Infantile Tremor Syndrom (ITS). ITS is a syndrome of tremor, mental and physical retardation, pigmentary changes of hair and skin and anemia in malnourished children. Though first reported in Indian subcontinent many identical cases were reported from around the world. Our case is a 15 month old child with generalized tremor, mild hepatosplenomegaly with features of grade II malnutrition including skin and hair changes. All the signs and symtoms of tremor improved after treatment with the World Health Organization (WHO) protocol for protein energy malnutrition (PEM) and administration of propranolol without any side effects.     

Cerebellar ataxia with recovery related to central pontine myelinolysis

Summary Development of severe ataxia and mild pyramidal signs without mental deterioration, tetraparesis or pseudobulbar palsy during recovery from withdrawal delirium and initial hyponatraemia are unusual clinical features consistent with central pontine myelinolysis. This diagnosis was confirmed by magnetic resonance imaging (MRI) in an alcoholic man. Clinical and electrodiagnostic improvement occurred, whereas the MRI findings remained unchanged in a follow-up study.     

Regressive dystonia and cerebellar ataxia: two unusual symptoms in central pontine myelinolysis

Two patients with central pontine myelinolysis who presented with dystonia are described. In one, it was associated with cerebellar ataxia which spontaneously improved. In the second, dystonia progressively disappeared 6 months later. In both cases magnetic resonance imaging (MRI) revealed characteristic pontine lesions. Extrapontine myelinolysis involving the putamen was also observed in one patient. Even when the basal ganglia seem to be spared on MRI, dystonia is probably due to their involvement by myelinolysis. Cerebellar ataxia may be related to peduncular or cerebellar lesions or both.     

Pathogenesis and prevention of central pontine myelinolysis: A case report

Central pontine myelinolysis (CPM) is a demyelinating disease producing serious or even fatal illness. The cause of the disease has been primarily attributed to alcoholism. Recently hyponatremia and its rapid correction have been related to CPM. We describe an alcoholic patient who developed the disease. The case has given us the opportunity of reflecting on CPM pathogenesis and management.

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Sommario La mielinolisi centrale del ponte è una malattia demielinizzante che può anche essere fatale. La sua causa è stata, in principio, riconosciuta nell;alcolismo. Recentemente l’iponatremia e la sua rapida correzione sono state messe in rapporto alle mielinolisi. In questa nota viene descritto un paziente alcolista cronico affetto da una forma lieve di mielinolisi centrale del ponte. L’osservazione di questo caso ha suggerito interessanti considerazioni patogenetiche e terapeutiche più generali.

     

Outcome of central pontine and extrapontine myelinolysis (n = 44)

The findings in 44 patients (42 of whom were chronic alcoholics) with central pontine myelinolysis show that the outcome does not depend on the severity of neurological deficits during the acute phase of the condition or on concomitant internal diseases, including the degree of hyponatremia. Of the 34 patients for whom follow-up data were available, 32 survived. Of these 11 completely recovered, 11 had some deficits but were independent, and 10 were dependent (4 through disorders of memory or cognition, 3 with tetraparesis, 2 with cerebellar ataxia, 1 with polyneuropathy). The electrophysiological findings did not contribute usefully to the prediction of outcome. Additional neuroradiological diagnostic testing with magnetic resonance imaging was also of no prognostic significance. The extent of the initial pontine lesion was not correlated with the severity of clinical findings during the acute phase of disease, nor was persistence of the pontine lesion as usually seen on magnetic resonance imaging correlated with clinical improvement. We conclude that patients with cerebral myelinolysis survive if the nonspecific secondary complications of transient illnesses such as aspiration pneumonia, ascending urinary tract infection with subsequent septicemia, deep venous thrombosis, and pulmonary embolism can be avoided. Received: 10 March 1998 Received in revised form: 18 January 1999 Accepted: 8 February 1999     

Central pontine myelinolysis in AIDS

Central pontine myelinolysis (CPM) is an uncommon complication in sick patients with severe underlying disorders such as chronic alcoholism, malignancy, malnutrition and hyponatraemia. We report two patients with advanced HIV infection who developed CPM. In one case the diagnosis was not suspected in life, in the other the diagnosis was made just before death, on the basis of magnetic resonance (MR) imaging appearances. At post mortem there was a close correlation between the MR abnormalities and the anatomic changes in the pons. Received: 6 March 1998 / Revised, accepted: 11 May 1998