Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023380 (lethargy)
 5,697 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A case, a 18-year-old male, of an endodermal sinus tumor (yolk sac tumor) in the fourth ventricle, was reported. The patient had a month history of headache, vomiting and gait disturbance prior to the hospitalization, when he admitted to our service he was in lethargic condition with left cerebellar ataxia and horizontal nystagmus. Lumbar tap revealed clear CSF under normal pressure of 110 mm H2O with the CSF protein of 432.5 mg/dl and cell count of 147/3. The vertebral angiography demonstrated space occupying lesion in the posterior fossa. Plain CT demonstrated only disappearance of the fourth ventricle and slightly dilated bilateral ventricles and third ventricle. However diffuse high density area around the fourth ventricle was demonstrated and the wall of bilateral anterior horn was slightly enhanced, after injection of contrast media. There was no other abnormal findings around the pineal region. Suboccipital craniectomy was performed and the tumor was totally removed macroscopically. The tumor was situated in th floor of the fourth ventricle and infiltrated into the fourth ventricular wall and th adjacent cerebellar tissue. The tumor was with soft, greyish color and extremely vascular. Histologically the tumor was diagnosed as endodermal sinus tumor according to Teilum's classification. There were stellate cells arranged in a loose with vacuolated network which formed cystic cavities and a complicated network of honeycomb appearance with a system of communicating cavities and channels. Various size of intra- and extracellular PAS-positive hyaline globules were also seen. Glomerular-like structure (Schiller-Duval body) was not observed. Immunoperoxidase study clearly demonstrated the presence of intra- and extracytoplasmic alpha-fetoprotein granules in the tumor tissue. The amount of the serum alpha-fetoprotein, measured by radioimmunoassay, showed 400 ng/ml. After irradiation in the posterior fossa (5000 rad) the patient was discharged. Three months later, follow up CT demonstrated small high density area in the anterior horn of the left lateral ventricle, so he was rehospitalised. Irradiation in the whole brain was again administered. The tumor was very radiosensitive. CT, after 800 rad, demonstrated complete disappearance of the tumor. After irradiation totally (3000 rad), he discharged with left cerebellar ataxia.
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PMID:[Primary endodermal sinus tumor of the fourth ventricle (author's transl)]. 616 17

A 17 year old high school boy experienced fever and diarrhea, which subsided within 4 days by appropriate medications. Six days later, however, he developed unsteadiness and limb spasm. On the morning of admission, he was found to have drowsiness, dysarthria, gait disturbance and involuntary jerks. When he was brought to the hospital, he was lethargic but could follow simple verbal commands. Frequent involuntary movements manifested by facial grimacings, limb spasms and twitchings with dystonic features were seen. Decorticate posturing was readily elicited by painful stimuli. There was no meningeal irritation sign or gross sensory impairment. The deep tendon reflexes were symmetrically exaggerated with bilateral Babinski signs. Bilateral lateral rectus muscle weakness was found together with mild ptosis and upward gaze limitation. Nystagmus was not present and the funduscopic examination was normal. Immediately he was placed on anticonvulsants, steroid hormone, gamma-globulin and antibiotics as well. A brain CT scan and a CSF examination revealed no abnormality. Meanwhile he continued to show a progressive deterioration associated with fever and status epilepticus, and within 24 hours he lapsed into coma in decorticate posture. An EEG obtained at the 3rd hospital day was compatible with spindle coma. In spite of aggressive treatment he remained febrile and comatous. Therefore, vidarabine (adenine arabinoside) was initiated from the 3rd hospital day for 5 days. Then he began to groan and show frequent choreic movements. For the subsequent 2 weeks he made a slow recovery.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of brain stem encephalitis with complete recovery (Bickerstaff's encephalitis)]. 620 73

Two female infants with nonketotic hyperglycinemia (NKH) were treated with diazepam for the control of seizures. The first infant had seizures, lethargy, and respiratory distress in the first 24 hours of life. The diagnosis of NKH was made at 3 weeks of age and she was then placed on a regimen of strychnine and a low-protein diet. Strychnine therapy was discontinued after three months of treatment because there was no improvement in the seizure control or in the patient's condition. At 5 months of age the patient was referred to our clinic for further work-up. The second infant had seizures, hypotonia, and respiratory distress shortly after birth. She was treated with phenobarbital and diphenylhydantoin, which had no effect on her seizures. The baby was referred to our clinic at 8 months of age and diagnostic studies revealed NKH. All previous medications were stopped and both infants were placed on diazepam, a competitor for glycine receptors in the CNS. Choline and folic acid were added for one-carbon unit transfer and sodium benzoate to bind excessive glycine. Both infants responded to this treatment with cessation of seizures; they became more responsive and alert, and their EEGs showed remarkable improvement despite the persistence of elevated glycine levels in plasma, CSF, and urine. Diazepam as a competitor for the receptors of glycine may prove helpful in controlling the intractable seizures associated with NKH.
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PMID:Nonketotic hyperglycinemia: treatment with diazepam--a competitor for glycine receptors. 630 Jul 46

The salient features of nonketotic hyperglycinemia include apnea, feeding difficulties, lethargy, seizures, abnormal muscle tone and reflex activity, significant developmental delay, and, in most instances, early death. The pathogenesis of the biochemical defect leading to increased glycine concentration in blood, urine, and CSF is likely to concern derangements of the glycine cleavage enzyme and/or transport mechanisms of glycine. Our current state of knowledge of this disorder is incomplete. Therapeutic attempts, as described in Table 2, have been largely unsuccessful. Further basic research on the underlying biochemical perturbation, including additional documentation of the glycine cleavage enzyme deficiency patterns, of substrate inhibition of key metabolic pathways, and of glycine transport aberrations, as well as investigations of new pharmacologic approaches, will be a challenge for investigators in this field. It is hoped that new knowledge in these areas will eventually lead to reduction of morbidity and mortality in children with nonketotic hyperglycinemia.
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PMID:Nonketotic hyperglycinemia: clinical, biochemical, and therapeutic considerations. 630 56

An American citizen acquired African trypanosomiasis while on a hunting safari in Sudan, East Africa. His travel history and rapid onset of symptoms, including fever, chills, headache, lethargy, and weight loss, were suggestive of Trypanosoma brucei rhodesiense infection, and trypanosomes were demonstrated in routine blood smears and buffy-coat preparations. Despite the presence of headaches, nuchal rigidity, and CSF pleocytosis, he was treated for non-CNS African trypanosomiasis, based on a normal CSF IgM level. This case report, along with a review of previously reported cases of imported African trypanosomiasis, illustrates the importance of clinical consideration of this rare, but often misdiagnosed, tropical illness in febrile patients returning from Africa.
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PMID:African trypanosomiasis in an American hunter in East Africa. 667 14

Nonketotic hyperglycinemia was diagnosed in identical twins with lethargy and respiratory failure in the neonatal period. Therapy with strychnine (0.32 mg/kg/day) resulted in great reductions in CSF and plasma glycine levels and improvement in muscle tone, respiration, and ability to suck. Myoclonic seizures were partially controlled by therapy with clonazepam. Higher dosages of strychnine (up to 2.0 mg/kg/day) were needed to counteract the increased lethargy following administration of clonazepam. At 5 months of age, the twins' developmental performance remained below the 1-month level despite adequate somatic growth. The twins died suddenly of status epilepticus at 6 1/2 months of age.
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PMID:Nonketotic hyperglycinemia. Effects of therapy with strychnine. 676 93

In 11 patients with complex partial epileptic seizures stuporous states were observed during treatment with valproate (VPA) (2 cases), with VPA and phenobarbitone (PB) (4 cases), or with VPA, PB and a third anti-epileptic drug (5 cases). Based on 3 characteristic cases, an attempt is made to define the role of VPA, the nature of the stuporous states, and the origin of digestive disorders which often herald the onset of behavioural disorders. Several clinical studies have suggested the direct responsibility of VPA even if the adverse effects are potentiated by many other anti-epileptic drugs. Stuporous states are not due to VPA overdose and do not depend on the mode of administration. No correlation has been found between electroclinical signs and plasma or CSF levels of the different anti-epileptic drugs. Reported data and the present cases suggest a paradoxical epileptogenic role for VPA on complex partial seizures: there exists a close similarity of electroclinical findings between spontaneous epileptic seizures and stuporous states during DPA treatment. Digestive disorders appear to result from a central mechanism and not from digestive tract intolerance. In some cases, it is likely that partial seizures with digestive symptoms and signs do occur.
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PMID:[Stuporous states during treatment with sodium valproate. Pathogenetic hypotheses]. 679 80

This study analyzes the clinical and bacteriologic features of CSF infections that occurred in patients with neoplastic meningitis treated with thiotepa and methotrexate administered through a subcutaneous reservoir and ventricular catheter (SRVC). Thirty-one patients were treated, and CSF infections occurred in four (13%). Staphylococcus epidermidis was the infecting organism in each case and Pseudomonas maltophilia occurred with S epidermidis in one patient. Fever, headache, lethargy, and evidence of CSF extravasation around the SRVC were the common manifestations of infection. The CSF leukocytosis was the only laboratory abnormality noted. All infections were cured with the appropriate antibiotics and removal of the SRVC. Risk of CSF infection did not seem to be related to the use of high doses of dexamethasone, cranial radiation therapy, or the presence or absence of leukopenia. The SRVCs were replaced and treatment of neoplastic meningitis was resumed in three patients; infection did not recur. A CSF infection during management of neoplastic meningitis may be treated effectively and does not preclude adequate therapy of neoplastic meningitis.
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PMID:CSF infections complicating the management of neoplastic meningitis. Clinical features and results of therapy. 680 96

Primary amebic meningoencephalitis and granulomatous amebic encephalitis are well recognized clinicopathological entities caused by free-living amebas. Associated arteritis and "mycotic aneurysms" with infiltration of intracranial arteries by lymphocytes, amebic trophozoites and cysts have not been previously reported. A 26-month-old girl had a 3-week history of encephalitis, characterized, initially, by vomiting and low-grade fever. Subsequently, she developed ataxia, generalized weakness, lethargy, and esotropia. The first CSF showed 490 RBC/microliters, 705 WBC/microliters with 90% mononuclears. Her pupils reacted briskly to light. Moderate nuchal rigidity, nystagmus, fixed downward gaze, anisocoria, bilateral 6th nerve palsy, left arm monoparesis and left Babinski were present. CAT scan revealed slight symmetrical dilatation of anterior horns of lateral ventricles and an area of abnormal enhancement above the 3rd ventricle. She died 14 days after admission, 5 weeks after onset of symptoms. The brain showed focal necrotizing encephalopathy, involving thalami, cerebellum, brain stem, and cervical and upper thoracic spinal cord. Numerous free-living amebic trophozoites and cysts were present within a chronic granulomatous encephalitis. There were trombosis of basilar, posterior cerebral, and vertebral arteries with profuse chronic panarteritis, fibrinoid necrosis, and mycotic aneurysms.
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PMID:Granulomatous encephalitis, intracranial arteritis, and mycotic aneurysm due to a free-living ameba. 689 86

Six infants with disseminated HSV had no mucocutaneous lesions at any time during the course of the illness. These infants presented with lethargy, poor feeding, apnea, acidosis, and hepatomegaly. The diagnosis of HSV was made by culturing the infant's oropharynx and blood, and the maternal cervix. Eight infants with HSV encephalitis had no skin, eye, or mucous membrane lesions. These infants presented with lethargy and low-grade fever, followed within 24 hours by the onset of focal partial motor seizures. The seizures were refractory to anticonvulsant therapy. The mean CSF white cell count was 131 cells/mm3;the glucose and protein concentrations were in the normal range. Brain biopsy was required for the early diagnosis of HSV encephalitis. These 14 cases presented 70% (14/20) of all infants with neonatal HSV diagnosed during the study period. HSV infection should be considered in infants with no mucocutaneous lesions who have signs usually associated with bacterial sepsis or who develop focal seizures during the first three weeks of life.
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PMID:Neonatal herpes simplex infection in the absence of mucocutaneous lesions. 706 32


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