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1.
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。 相似文献
2.
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. 相似文献
3.
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. 相似文献
4.
Recovery after central pontine myelinolysis 总被引:2,自引:0,他引:2
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.相似文献
5.
Mochizuki H Masaki T Miyakawa T Nakane J Yokoyama A Nakamura Y Okuyama K Kamakura K Motoyoshi K Matsushita S Higuchi S 《Journal of neurology》2003,250(9):1077-1083
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. 相似文献
6.
Hans Walter Pfister Karl Max Einhäupl Thomas Brandt 《European archives of psychiatry and clinical neuroscience》1985,235(3):134-139
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. 相似文献
7.
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.相似文献
8.
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. 相似文献
9.
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. 相似文献
10.
Regressive dystonia and cerebellar ataxia: two unusual symptoms in central pontine myelinolysis 总被引:1,自引:0,他引:1
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. 相似文献
11.
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 相似文献
12.
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. 相似文献
13.
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.相似文献
14.
R. F. Miller Michael J. G. Harrison Margaret A. Hall-Craggs Francesco Scaravilli 《Acta neuropathologica》1998,96(5):537-540
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 相似文献
15.
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 相似文献
16.
Kobayashi Junpei Nagao Masahiro Kawata Akihiro Matsubara Siro 《Journal of neurology》2009,256(8):1369-1371
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. 相似文献
17.
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 相似文献
18.
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. 相似文献
19.
20.
卢岩 《神经疾病与精神卫生》2010,10(3):322-324
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. 相似文献