Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0038220 (status epilepticus)
 7,272 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A previously healthy 8-month-old girl developed exanthem subitum and acute encephalopathy with status epilepticus, quadriplegia and bilateral abducens nerve palsies. Human herpesvirus-6 DNA was found in the cerebrospinal fluid by the polymerase chain reaction at the acute stage. Cranial computed tomography showed low density areas in the thalami and in the cerebellar and abducens nuclei. The distribution of the lesions was consistent with acute necrotizing encephalopathy. As for the thalamic lesions, a T2 weighted magnetic resonance image on the 24th day of the illness demonstrated low signal intensity surrounded by high intensity; 99mTc-ECD SPECT showed hypoperfusion, which suggested irreversible tissue damage. The patient is now 1 year 6 months old and has spastic quadriparesis with mental retardation and abducens nerve palsies.
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PMID:Serial neuroimages of acute necrotizing encephalopathy associated with human herpesvirus 6 infection. 857 24

Six patients (4 boys and 2 girls) with hemiconvulsion-hemiplegia-epilepsy (HHE) syndrome are described. They had prolonged seizures, lasting from 30 min to 12 h, at ages 1-4 years. These took the form of hemiconvulsion in three of the children and generalized tonic-clonic seizures in the others, being preceded by hemifacial twitching or head and eye deviation in two. They were followed by hemiplegia, which cleared with time in five patients, apart from subtle pyramidal tract signs. One child had spastic quadriparesis, choreiform movements, contracture deformities and severe mental retardation following repeated status epilepticus. Subsequent epilepsy developed in five patients and was satisfactorily controlled with carbamazepine and/or phenobarbitone. Cerebral hemiatrophy was documented in all patients by cranial computed tomography and/or magnetic resonance imaging. Single photon emission computed tomography (done in 4 patients) showed ipsilateral hypoperfusion (of the damaged hemisphere). Electroencephalography showed ipsilateral slowing and low voltage of background activity. Epileptiform discharges were found on the ipsilateral side in two cases and the contralateral side (the undamaged hemisphere) in one.
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PMID:Hemiconvulsion-hemiplegia-epilepsy syndrome. A clinical, electroencephalographic and neuroradiological study. 922 14

A fatal case of acute intermittent porphyria in a 22 years old Indian male is reported. He presented with abdominal pain and constipation, subsequently developed status epilepticus, acute respiratory failure and quadriparesis. He succumbed to the illness on the twelfth day. Among the neuromuscular causes of acute respiratory failure, requiring ventilatory support, porphyria is a condition potentially treatable, but rarely suspected.
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PMID:Acute intermittent porphyria as a cause of acute respiratory failure. 1220 44

We report the first case of an autoimmune thyroid encephalopathy presenting with multifocal motor status epilepticus. A 37-year-old female patient was admitted with multifocal motor seizures intractable to intravenous status epilepticus treatments, asymmetrical quadriparesis, truncal ataxia and continuous semi-rhythmical jerks. Pathological signal alterations were detected in both precentral cortices in MRI examination. Autoimmune thyroiditis was diagnosed after radiological examinations of the thyroid gland and thyroid function tests. Seizures promptly ceased following intravenous steroid treatment. Immunohistochemistry studies showed mild to moderate neuronal staining with the plasma and CSF samples. Remarkably, autoimmune thyroiditis may present with migrating focal motor status epilepticus. We recommend anti-thyroid antibody screening for multifocal motor status epilepticus cases of unspecified cause.
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PMID:Autoimmune thyroid encephalopathy presenting with epilepsia partialis continua. 1692 5

Major abdominal surgeries, including liver transplantation, are considered high-risk procedures for patients with respiratory muscle dysfunction, such as patients with quadriparesis, due to possible fatal postoperative pulmonary complications. We report on a 57-year-old male patient with longstanding quadriparesis due to fifth cervical spine injury from a traffic accident who suffered from decompensated liver cirrhosis related to hepatitis C infection and hepatocellular carcinoma. A preoperative pulmonary function test showed forced expiratory volume in 1 minute (FEV1) 1.06 L, which was a risk for pulmonary complications. The patient required respiratory training. Cadaveric liver transplantation was performed successfully without surgical complications. The patient was extubated on the fourth day after surgery and initially did well. However, on the eighth postoperative day, an episode of status epilepticus from metabolic derangement developed. After controlling seizure with anticonvulsive medication and sedation, the patient was reintubated due to hypoventilation. Chest radiograph showed upper lung atelectasis. Due to this complication, tracheostomy was performed. The patient's condition gradually improved. He was ultimately discharged on the 45th postoperative day. Two months after the transplantation, liver functions were normal and the patient could breathe spontaneously without tracheostomy and had good quality of life. In conclusion, this is, to our knowledge, the first report of liver transplantation in a patient with quadriparesis. It shows that even with a very high risk for postoperative pulmonary complications, liver transplantation can be performed successfully with careful patient selection and effective respiratory care.
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PMID:Successful liver transplantation in a patient with quadriparesis: a case report. 2476 3