Dystonia in central pontine myelinolysis without evidence of extrapontine myelinolysis.

A 44-year-old female is described who developed persistent upper extremity and orolingual dystonia several weeks after suspected onset of central pontine myelinolysis (CPM), later confirmed by characteristic pontine lesions on MRI. No foci of the extrapontine myelinolysis were evident. This case confirms that dystonia may be a late and persistent sequela of CPM and may occur in the absence of visible lesions outside the brainstem.     

Magnetic resonance imaging in central pontine myelinolysis.

Magnetic resonance imaging (MRI) was performed in two patients in whom a clinical diagnosis of central pontine myelinolysis (CPM) had been made. MRI showed lesions in the pons in both cases about 2 years after the illness, at a time when the spastic quadriparesis and pseudobulbar palsy had recovered. The persisting abnormal signals in CPM are likely to be due to fibrillary gliosis. Persistence of lesions on MRI means that the diagnosis of CPM may be electively, after the acute illness has resolved.     

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.

     

Idiopathic central pontine myelinolysis in childhood

Central pontine myelinolysis (CPM) is rare in childhood with only a few cases reported in world literature. We report a 7-year-old male who presented with acute ataxia, swallowing difficulties, dysarthria, and radiological features consistent with the disorder. He improved remarkably with oral prednisolone therapy and was almost back to normal by 2 weeks. A review of the literature is also included.     

Computerized tomography in central pontine myelinolysis

We describe an autopsy-proven case of central pontine myelinolysis (CPM) with premortem computerized tomographic (CT) visualization of the lesion on two scans, performed with an interval of 2 weeks. This case demonstrates the capability of CT to support the clinical diagnosis of central pontine myelinolysis. Identification of the condition should facilitate prompt initiation of aggressive supportive care.     

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.     

Neuropsychiatric and neuropsychological manifestations of central pontine myelinolysis.

A patient with central pontine myelinolysis (CPM) underwent neurological and mental status examination, as well as neuropsychological testing, during the acute stage of the disease. After correction of the hyponatremia, a gross change in his neuropsychiatric status was observed. The patient underwent extensive neurological, psychiatric, and neuropsychological testing during the acute phase of the disease and at follow-up 4 months later. All major neurological and neuropsychiatric symptoms present at onset were fully reversible. Neuropsychological examination revealed deficits in the domains of attention and concentration, short-term memory and memory consolidation, visual motor and fine motor speeds, and learning ability. Although improved, neuropsychological testing still revealed remarkable deficits at follow-up. We conclude that neuropsychological deficits can accompany CPM, and that these deficits do not necessarily diminish simultaneously with the radiological or clinical neurological findings but may persist for a longer period of time, or even become permanent. In his recovery the patient started to manifest new neurological symptoms consisting of a mild resting tremor of both hands and slow choreoathetotic movements of the trunk and the head, which we considered to be late neurological sequelae of CPM. The significance of CPM in the differential diagnosis of acute behavioral changes after correction of hyponatremia is stressed, even if correction is achieved slowly and carefully.     

Behavioral manifestations of central pontine myelinolysis

A young woman with a clinical history and magnetic resonance imaging scan consistent with central pontine myelinolysis came to medical attention because of prominent behavioral symptoms. Marked clinical recovery occurred despite persistent radiologic abnormalities. Rapid correction of hyponatremia was probably related to the development of the central pontine myelinolysis. A normal computed tomographic scan and the absence of brain-stem signs delayed accurate diagnosis.     

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.     

Subclinical central pontine myelinolysis following liver transplantation

Central pontine myelinolysis (CPM) is a demyelinating disorder of unknown origin that almost exclusively affects the central portion of the basis pontis, and is one of the fatal neurological complications after liver transplantation. We describe two children with CPM detected incidentally after liver transplantation. To our knowledge, this is the first report of CPM diagnosed antemortem in children who had undergone liver transplantation. In our patients, there were no clinical manifestations associated with CPM. We conclude that, including silent cases such as our patients, CPM may be more prevalent than previously appreciated following liver transplantation.     

Peduncular hallucinosis secondary to central pontine myelinolysis

Peduncular hallucinations are generally associated with lesions in the midbrain. They have rarely been associated with central pontine myelinolysis, a condition associated with rapid alterations in serum sodium and chronic alcoholism. Described herein is the case of a 46‐year‐old man who developed typical peduncular hallucinations, whose imaging demonstrated central pontine myelinolysis. After alcohol cessation and neuroimaging resolution, the patient's hallucinatory phenomena abated.     

Parkinsonism and dystonia in central pontine and extrapontine myelinolysis

Parkinsonism as well as dystonic signs are rarely seen incentral pontine myelinolysis and extrapontine myelinolysis. A 51yearold woman developed central pontine myelinolysis and extrapontine myelinolysis with parkinsonism after severe vomiting which followed alcohol and drug intake, even though marked hyponatraemia had beencorrected gradually over six days. Parkinsonism resolved four monthsafter onset, but she then exhibited persistent retrocollis, spasmodicdysphonia, and focal dystonia of her left hand. Although the medicalliterature documents three similar patients, this patient is differentas dystonic symptoms only developed four months after parkinsoniansigns had resolved.

     

Association of central pontine myelinolysis and Marchiafave-Bignami disease

Combined pathologic features of central pontine myelinolysis and Marchiafava-Bignami disease occurred in a 53-year-old man with chronic alcoholism. This association is rare despite the basic similarities of these two disorders. The combination of both conditions in the same patient suggests a common pathogenetic mechanism.