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Query: UMLS:C0036572 (seizures)
 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We reviewed the clinical histories and autopsy records of 35 pediatric patients (ranging in age from 9 months to 18 years) who underwent orthotopic liver transplantation using ciclosporin and corticosteroids for immunosuppression. At the time of death, 19 children (54%) had encephalopathy, 16 (46%) were lethargic or in coma, 10 (29%) had seizures, and 10 were normal. Neuropathological lesions were found on postmortem examination in all 35 patients. Vascular lesions such as infarction, ischemia, thrombosis, and hemorrhage were the most common neuropathological findings (86%) followed by infectious processes (29%). Candida albicans (2 patients) and Aspergillus fumigatus (3 patients) were the only offending organisms identified, both causing meningoencephalitis. Alzheimer type II astrocytes, a characteristic feature of chronic liver disease, were the single most common autopsy finding (69%). Central pontine myelinolysis was seen in 3 children and basilar artery thrombosis affected 1 child. Neurological complications and their subsequent neuropathology are a significant cause of morbidity and mortality after pediatric liver transplantation. Vascular insults, electrolyte abnormalities, and infections that involve the central nervous system are directly related to liver function and the immunosuppression necessary to maintain graft viability. Only with continued observation after surgery combined with rapid medical and surgical treatment can we hope to improve the prognosis following liver transplantation in the pediatric population.
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PMID:Neuropathology of pediatric liver transplantation. 248 84

The clinical and neuropathologic findings of 55 adults and 30 children who received liver transplants were reviewed. Encephalopathy was the most common clinical neurologic syndrome and was usually caused by metabolic or anoxic causes. (Alzheimer type astrocytes were present in 73 per cent of patients, and evidence of diffuse hypoxic damage was present in 40 per cent of children and 25 per cent of adults.) Cerebrovascular lesions were a common finding with infarcts or hemorrhages present in 30 per cent of patients. CNS infections were documented in 34 per cent of patients. Seizures were present in a third of patients. Central pontine myelinolysis was present in 12 per cent of patients and was more common in adults than in children. Antemortem diagnosis of neurologic complications was more often based on clinical presentation rather than specific radiologic or laboratory tests.
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PMID:Neurologic complications of liver transplantation. 304 44

In summary, we believe that the osmotic difference between the brain cell and the extracellular fluid is the critical parameter in determining therapy. A gradient of 30 mosm/kg or more produces significant shifts of intracellular water and cell damage. Seizure and coma are the neurologic signs associated with acute life-threatening osmotic imbalance between swelling brain cells and the extracellular space. Treatment is designed to decrease this osmotic gradient to less than 30 mosm/kg to prevent this cell swelling. Thus in acute symptomatic hyponatremia, treatment (fluid restriction alone if urine osmolality is less than 100 mosm/kg or 3% saline if needed or both) is needed to decrease this osmotic gradient. If hyponatremia is chronic and brain osmotic adaptations have already taken place, increased extracellular osmolality would cause brain cells to undergo water loss, which may result in cell shrinkage and neurologic damage (for example, central pontine myelinosis). Unfortunately, there is as yet no clinically available tool to rapidly assess intracellular brain osmolality. Clinical judgment is therefore mandatory in trying to estimate the osmotic gradient between the intracellular and extracellular environment. Appropriate treatment must be initiated to prevent excessive changes in cell volume.
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PMID:Symptomatic hyponatremia: pathophysiology and management. 315 33

Patients with renal failure may manifest a variety of neurologic disorders. Patients with chronic renal failure who have not yet received dialytic therapy may develop a symptom complex progressing from mild sensorial clouding to delirium and coma, with tremor, asterixis, multifocal myoclonus, and seizures. After the institution of adequate maintenance dialysis therapy, patients may continue to be afflicted with more subtle nervous dysfunction, including impaired mentation, generalized weakness, and peripheral neuropathy. These central nervous system disorders are referred to as uremic encephalopathy. The dialytic treatment of end-stage renal disease has itself been associated with the emergence of two distinct, new disorders of the central nervous system; dialysis dysequilibrium and dialysis dementia. The dialysis disequilibrium syndrome consists of headache, nausea, muscle cramps, obtundation, and seizures, and is a consequence of the initiation of dialysis therapy in some patients. Dialysis dementia is a progressive, generally fatal encephalopathy which affects patients on chronic hemodialysis. There are at least three different forms of dialysis encephalopathy: sporadic, epidemic; and that associated with renal disease in children. In addition to the foregoing neurologic diseases which are specifically related to uremia and/or dialysis, a number of other neurologic disorders occur with increased frequency in patients with end-stage renal disease on chronic hemodialysis. These include subdural hematoma, electrolyte disorders, vitamin deficiencies, drug intoxication, hypertensive encephalopathy, and acute trace element intoxication. Renal transplantation is associated with a variety of central nervous system infections, reticulum cell sarcoma, and central pontine myelinosis. The present manuscript will review the clinical, structural, and biochemical components of those neurologic disorders which are peculiar to the uremic state and its treatment with dialysis.
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PMID:Uremic encephalopathies: clinical, biochemical, and experimental features. 675 30

Central pontine myelinolysis (CPM) is a demyelinating condition of the central pons with or without associated foci of demyelination in extrapontine areas. We present a case of partial ornithine carbamoyl transferase deficiency in a 5-year-old girl which was complicated by CPM. The patient was a previously undiagnosed girl who presented with mild hyperammonemic encephalopathy with a maximum plasma ammonia level of 376 microM on admission. Laboratory testing established the diagnosis of OCT deficiency, and therapy with hydration and protein restriction was successful in returning the plasma ammonia levels to normal. Five days after correction of her hyperammonemia, the patient developed intractable seizures and coma. Serial MRI scans of the brain revealed the evolution of the characteristic findings of CPM. Plasma ammonia and electrolyte concentrations were well controlled throughout this time. This represents the first description of CPM in a patient with a urea cycle defect.
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PMID:Central pontine myelinolysis as a complication of partial ornithine carbamoyl transferase deficiency. 757 73

Central pontine myelinolysis (CPM), a disorder occurring for the most part in alcohol abusers, is characterized by the symmetric and selective destruction of medullated sheaths in the central pontine region. Detection of CPM in the pre-symptomatic phase may improve the outcome, which is usually serious. We describe a case of CPM in a 48-year-old female alcohol abuser who was seen at our out-patient clinic for a history of generalized seizures. Neurological examination was normal while magnetic resonance imaging revealed a pontine lesion consistent with CPM. On the basis of this report we hypothesize that the possibility to detect CPM in the pre-symptomatic phase will lead to increased diffusion of magnetic resonance imaging, and in turn to improved therapeutic strategies, such as the use of greater caution when correcting plasma electrolyte imbalance in alcoholics.
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PMID:Central pontine myelinolysis: report of an asymptomatic case. 772 8

Neurological complications are common in cirrhotic patients with end-stage liver failure. They comprise a wide array of etiologies, which may originate before, during, or after liver transplantation. The objective of this study was to describe the nature of the main neurological complications in patients with end-stage liver failure. Several toxins including ammonia, manganese, benzodiazepine-like substances, gamma-aminobutyric acid-like substances, and impaired dopaminergic neurotransmission are at the top of the list of candidates for hepatic encephalopathy, subclinical encephalopathy, and extrapyramidal signs before liver transplantation. Central pontine myelinolysis, cerebrovascular autoregulation impairment, and paradoxical cerebral embolism are probably responsible for the neurological complications during liver transplantation. Neurological complications represented by alterations of mental status, seizures, and focal motor deficits have been described after liver transplantation. These complications have been attributed to several pathogenetic factors, such as a poorly functioning graft, an intracranial hemorrhage, a cerebral infarction, an infection, or the toxicity of immunosuppressants.
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PMID:Neurological complications of liver cirrhosis and orthotopic liver transplant. 1664 71

Central pontine myelinolysis and extrapontine myelinolysis are rare complications of rapid correction of chronic hyponatremia. Central pontine myelinolysis is more common and more predictable in presentation. Extrapontine myelinolysis' presenting symptoms vary and may be as general as lethargy or altered mood. We report experience with a patient who developed only extrapontine myelinolysis after rapid correction of severe hyponatremia associated with gastroenteritis. His presenting sign, generalized seizures, has not been previously reported. We summarize risk factors for myelinolysis, which may be more common than previously thought, as well as steps to minimize risk while managing vulnerable patients.
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PMID:Extrapontine myelinolysis after correction of hyponatremia presenting as generalized tonic seizures. 1937 39

Central pontine myelinolysis (CPM) has been reported in women with severe hyperemesis gravidarum-induced hyponatremia followed by rapid correction. Gestational diabetes with adipsia complicated by acute hypernatremia resulting in CPM has never been reported. Here is a case of a disabled female who presented with polydipsia, polyuria, seizures, fetal death in utero, hyperglycemia, and hyper-osmolar hypernatremia on her 31st gestational week. The dead fetus was delivered and the patient's plasma glucose and sodium were later stabilized. When the patient developed quadriplegia and respiratory failure 5 days later, brain magnetic resonance imaging showed central pontine and extra-pontine myelinolysis. Gestational diabetes complicated by hyper-osmolar crisis may cause fetal death and severe neurologic sequela. Early recognition and delivery of the fetus and placenta may improve the electrolyte and fluid imbalance.
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PMID:Gestational diabetes and central pontine myelinolysis with quadriplegia: a case report. 1965 45

Central pontine myelinolysis (CPM) is a demyelinating disorder, which is associated most commonly with the rapid correction of hyponatraemia and other abrupt changes in physiological osmotic conditions. This includes the treatment of hyperosmolar hyperglycaemia in diabetes mellitus (DM) sufferers. Herein, we report a case of CPM in a 55-y-old patient with new-onset DM who presented with partial focal seizures and a sudden-onset right-sided hemiplegia. Magnetic resonance imaging revealed a lesion in the central pons. The patient responded to glucose control and antiepileptic medication, and achieved a recovery of limb function within 17 d of admission. CPM occurred in this patient before the correction of hyperglycaemic hyperosmolar state, and a disturbance in his initial electrolytes was not found. This report is the first documented case of long-term hyperglycaemic hyperosmolar state leading to CPM, and highlights that CPM can present as an isolated hemiplegia.
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PMID:Central pontine myelinolysis in a patient with epilepsia partialis continua and hyperglycaemic hyperosmolar state. 2109 47


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