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。     

Brain-stem auditory evoked potentials and blink reflex in friedreich's ataxia

Summary The brain-stem involvement in Friedreich's ataxia (FA) was studied by using brain-stem auditory evoked potentials (BAEPs) and the blink reflex. Ten out of 18 patients had abnormal BAEPs, the main abnormality being complete absence of responses and disappearance of wave V. Combined degeneration of the peripheral and central acoustic pathways probably accounts for these findings. The blink reflex was abnormal in 50% of the cases. The outstanding abnormality was bilateral delay of late responses with normal early response, which could be correlated with the known pallor of the descending trigeminal tracts. In contrast with BAEP findings, blink reflex abnormalities did not correlate with either the age of patients or the severity and duration of the disease. These data suggest a difference in susceptibility to degeneration between the auditory system and neuronal system subserving the blink reflex. We conclude that systematic BAEP and blink reflex recording is useful in the electrophysiological evaluation of FA patients.     

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.

---

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.

     

Benign type of central pontine myelinolysis in alcoholism

Nine alcoholic patients with central pontine myelinolysis (CPM),who showed a favorable prognosis, are reported. The majority of them had taken part in binge drinking and had a subsequent consciousness disturbance for 18.1±10.9 (mean±SD) days. None of the patients had had acute correction of hyponatremia. Truncal ataxia and gait instability were present in most of the patients after recovery from the disturbance of consciousness. Most of them eventually gained independence, and magnetic resonance imaging showed that their pontine lesions tended to shrink. Electrophysiological studies detected prolonged latency between the I and III waves in auditory brainstem responses and between N11 and P13/14 onsets in the somatosensory evoked potentials. These clinical, radiological and electrophysiological findings should be of use in diagnosing CPM.     

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.     

Central pontine myelinolysis: Diagnosis by computed tomography, magnetic resonance and evoked potentials

The intravital diagnosis of central pontine myelinolysis has become possible with the introduction of computed tomography and magnetic resonance into neurological diagnostics. These tools are of special value when neurological signs of a ventral pontine lesion are lacking, as in the case we describe. Auditory evoked potentials likewise confirm their diagnostic value with regard both to the site of the lesion and to its dorsal extent toward the pontine tegmentum.

---

Sommario La possibilità di una diagnosi intra-vitam della mielinolisi pontina centrale è diventata una realtà con la introduzione della tomografia computerizzata e della risonanza magnetica nella diagnostica neurologica. Questi mezzi diagnostici acquistano un particolare valore nei casi in cui mancano i segni neurologici riferibili ad una lesione ventrale pontina, come nel caso clinico qui illustrato. I potenziali evocati uditivi confermano anche essi la loro utilità diagnostica sia per quanto riguarda la sede della lesione, sia per quanto riguarda la estensione dorsale verso il tegmento pontino.

     

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.     

Brain-stem involvement in multiple sclerosis: a comparison between brain-stem auditory evoked potentials and the acoustic stapedius reflex

Summary Brain-stem auditory evoked potentials (BAEPs) and the acoustic stapedius reflex (ASR) were recorded in 68 patients with definite, probable and possible multiple sclerosis (using the definitions of McAlpine). The high incidence of abnormal results, 68% and 60%, respectively, pointed to the diagnostic value of these two measures in detecting brain-stem dysfunction. Combination of the methods increased the diagnostic yield to 85%. Since in part the same brain-stem generator sites underlie BAEPs and the ASR, it was considered that a study of their correlation might serve to increase the reliability and validity of these techniques. There was 71% agreement overall between results from the two measures. Furthermore, 72% of the joint BAEP and ASR abnormalities corresponded in detection of the brain-stem lesion site. It was concluded that the combined approach may supply powerful, complementary information on brain-stem dysfunction, which may aid in establishing the diagnosis of multiple sclerosis.This study was supported by the M. Sackler Foundation for Multiple Sclerosis Research     

Central pontine myelinolysis

Summary Thirty-seven cases of central pontine myelinolysis (CPM) with miscellaneous underlying disorders were found in 1,000 consecutive autopsies, of which 636 brains were examined. The incidence of CPM in this study was 5.8%. The frequent underlying disorders were malignant neoplasms (43%), chronic pulmonary disease (27%), and chronic renal failure cases under dialysis treatment (14%). Fatty liver cirrhosis due to alcohol abuse was recognized in only one CPM case. In the present study, 78% of the CPM cases revealed either electrolyte disturbances or abnormal blood gas data, such as marked deviation of base excess and/or of serum pH in 62.5%, hyper- or hypochloremia (above 115 mEq/l, below 95 mEq/l) in 47%, hyper- or hyponatremia (above 150 mEq/l, below 130 mEq/l) in 25%, marked hypoxemia (less than 40 mmHg) in 12.5% and hypokalemia (below 3.0 mEq/l) in 9% of the CPM cases. The myelinolytic changes were localized in the basis pontis in 14 of 37 CPM cases and in the basis pontis and the cerebral and/or cerebellar white matter (extrapontine myelinolysis) in the remaining 23 cases. The extrapontine changes were also closely related to the electrolyte disturbances or the abnormal blood gas data. The results of this study suggest that myclin and oligodendrocytes in the basis pontis and cerebral and cerebellar white matter are vulnerable to abnormal levels of serum electrolytes and also to marked changes of the acid-base balance.     

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.

---

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.

     

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     

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     

A case of late adult-onset dentatorubral-pallidoluysian atrophy mimicking central pontine myelinolysis

We report the case of a 52-year-old man with late-onset dentatorubral-pallidoluysian atrophy (DRPLA). MRI findings of late-onset DRPLA usually showed the involvement of cerebral white matter lesions with high intensity on T2-weighted images (WI), in addition to brainstem, globus pallidus, and thalamus. But our patient did not present with abnormal manifestation of white matter lesions of the cerebrum. In addition, the appearance of pontine base was remarkably similar to central pontine myelinolysis (CPM). There is no reported case of DRPLA mimicking CPM in the literature, while there is one previous report of CPM with cerebellar ataxia without pyramidal tract involvement, and CPM may exhibit cerebellar ataxia. Although there is differentiation between CPM and DRPLA by the presence of the atrophy of brainstem and cerebellum, the characteristic MRI findings of pontine base may make it difficult to differentiate CPM with cerebellar ataxia from DRPLA with inconspicuous leukoencephalopathy. In such a situation, we should return to the clinical history and background of a patient, and, if necessary, DNA analysis should be performed for a definite diagnosis.     

酒精所致精神障碍患者的脑干听觉诱发电位

目的分析酒精所致精神障碍患者的脑干听觉诱发电位（ＢＡＥＰ）表现。方法对３０例长期嗜酒所致精神障碍患者进行ＢＡＥＰ检测，并与正常人进行对照。结果病例组ＢＡＥＰ各波峰潜伏期（ＰＬ）、峰间期（ＩＰＬ）延长，波形分化差，与正常对照组有显著性差异。结论ＢＡＥＰ异常与临床表现及影像学，实验室结果基本一致，提示诱发电位可作为慢性酒精中毒的诊断方法之一     

Brain function in patients with cerebral fat embolism evaluated using somatosensory and brain-stem auditory evoked potentials

Summary In two victims of traffic accidents with broken bones and fat embolism, serial recordings of somatosensory evoked potentials (SEPs) and brain-stem auditory evoked potentials (BAEPs) were examined to assess brain function. Initial SEPs and BAEPs revealed normal subcortical components, while the late cortical components of SEPs were abolished, findings indicative of diffuse dysfunction of grey rather than of white matter. As the neurological functions became normal, the late components appeared. It is concluded that while absent late components of SEPs do reflect cortical dysfunction, they are not necessarily associated with a poor prognosis. Repetitive recordings of SEPs appear to be a useful tool for assessing the neurological condition and the prognosis of patients with cerebral fat embolism.     

1例缓慢补钠致脑桥中心／脑桥外髓鞘溶解症分析

目的 提高对危重患者救治过程中发生脑桥中央髓鞘溶解症（CPM）/脑桥外髓鞘溶解症（EPM）的认识.方法 对1例危重患者救治过程中发生CPM/EPM的临床资料、辅助检查及相关文献进行分析.结果 对营养不良、严重感染伴有低钠血症的患者,每日纠正血钠10mmol/L亦可发生渗透性髓鞘溶解症,核磁检查显示病灶更清晰.结论 慢性低钠血症患者为避免出现CPM/EPM,每日血钠纠正应＜10mmol/L.