Cases reported "Chronic Disease"

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1/438. Latent coeliac disease. Personal experience.

    Latent coeliac disease (L.C.D.) is an extremely rare condition to describe. In this study we analyzed three cases of patients affected by L.C.D.: two of them suffered from insulin-dependent diabetes mellitus (IDDM) and the other one from infantile cerebral palsy and eosinophilic gastroenteritis. We confirm the existence of this form of coeliac disease (C.D.), by means of duodenal biopsy, and stress the importance of an early diagnosis in order to prevent the serious consequences caused by untreated C.D.
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ranking = 1
keywords = cerebral
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2/438. central nervous system T-cell lymphoproliferative disorder in a patient with chronic active Epstein-Barr virus infection.

    PURPOSE: central nervous system (CNS)-T cell lymphoproliferative disorder (T-LPD) developing during the course of chronic active Epstein-Barr virus (CAEBV) infection is reported. patients AND methods: CAEBV was diagnosed in a 14-month-old boy with fever, cytopenia, hepatosplenomegaly, and abnormal high titers of anti-Epstein-Barr virus (EBV) antibodies. At 8 years of age, he had a splenectomy because of progressive disease. RESULTS: After 27 months of clinical remission, muscle weakness and paresthesia developed. magnetic resonance imaging of his brain showed spotty T2 prolongation in left parietal, bilateral frontal, and temporal white matter with meningeal enhancement. Brain biopsy revealed the cerebral infiltration of CD3 , CD4 , CD8-, CD45RO , CD56-, and EBV-encoded rna 1 cells. CONCLUSIONS: The CNS involvement of EBV-associated T-LPD is a rare but serious complication in CAEBV without known underlying immunodeficiency.
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ranking = 1.0583303060737
keywords = cerebral, brain
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3/438. The effect of lidocaine on chronic tinnitus: a quantitative cerebral perfusion study.

    The cerebral perfusion of a 55-year-old female patient with chronic tinnitus was investigated quantitatively by single photon emission computed tomography (SPECT) with the xenon133 inhalation method. The first investigation was performed under standard conditions, the second during suppression of tinnitus with intravenous injection of lidocaine. As a reference a healthy volunteer was included under the same conditions. The global cortical perfusion was identical on both sides except for increased perfusion in the auditory cortex, especially on the right side when tinnitus lasted during the first measurement. The second investigation under treatment with lidocaine and during the tinnitus-free interval showed a decrease of the global cortical perfusion with a slightly higher value at the temporal cortex bilaterally. In comparison to the first investigation, a difference between right and left auditory cortex could no longer be demonstrated, and also the difference between regional and global perfusion decreased. A cerebral effect under treatment of tinnitus with lidocaine was shown by the quantitative measurement of brain perfusion. It is debatable whether this effect on cerebral activity results from the medication or as a reaction to the tinnitus-free interval.
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ranking = 7.0583303060737
keywords = cerebral, brain
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4/438. Internuclear ophthalmoplegia following minor head injury: a case report.

    Internuclear ophthalmoplegia (INO) is a common sign of multiple sclerosis in young patients and of vascular diseases in older people. Traumatic bilateral internuclear ophthalmoplegia following severe head injuries may occur. We present the unusual case of a young patient suffered from bilateral INO as an isolated finding after a minor head injury, without other signs of brain stem or cortical injury. The ophthalmoplegia has persisted for 22 months.
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ranking = 0.058330306073714
keywords = brain
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5/438. Calcified chronic subdural hematoma: case report.

    Calcified or ossified chronic subdural hematoma is a rare entity that usually presents as a space-occupying lesion over the cerebral convexity. We report a case of calcified and ossified chronic subdural hematoma in an unusual location that has not been previously reported. A 24-year-old man with a history of tonic-clonic convulsions since 7 months of age was admitted because of increasing frequency and duration of seizures. Computed tomography and magnetic resonance imaging demonstrated a fusiform extra-axial lesion just above the tentorium and adjacent to the cerebral falx. A calcified and ossified chronic subdural hematoma was noted and was almost completely removed by craniotomy. Better seizure control was achieved by removal of the calcified chronic subdural hematoma. Calcified subdural hematoma, calcified epidural hematoma, calcified empyema, meningioma, calcified arachnoid cyst, and calcified convexity of the dura mater with acute epidural hematoma should be considered for the differential diagnosis of an extra-axial calcified lesion.
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ranking = 2
keywords = cerebral
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6/438. Chronic spinal subdural haematoma associated with intracranial subdural haematoma: CT and MRI.

    Chronic spinal subdural haematoma is a uncommon. We describe the CT and MRI appearances of chronic spinal and intracranial subdural haematomas following minor trauma. The aetiology, pathogenesis and differential diagnosis are discussed.
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ranking = 46.294680667447
keywords = haematoma
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7/438. Sibling cases of chronic recurrent hepatocerebral disease with hypercitrullinemia.

    Two sibling cases with chronic recurrent hepato-cerebral syndrome which correspond to the nutritional form of hepato-cerebral disease entitled by Shikata et al. and the data of plasma free aminoacids analyses of these cases were reported. The one case is 27 years old male and the other case is 36 years old female. Their parents were cousins. Both cases have had unbalanced diet, especially liked legumes unusually. Their main symptom was recurrent disturbance of conciousness and convulsive seizures. Slight abnormality of liver function test and hyperammonemia were demonstrated. Electroencephalogram showed the pattern of triphasic wave. Coeliac angiography did not revealed a portal-systemic shunt. Hepatic biopsy specimen revealed liver fibrosis with fatty change in the one case and mild fatty change in the other case. Analyses of plasma free aminoacids showed particurally high level of citrulline in both cases. From the results of plasma free aminoacids analyses, it is considered that pathogenesis of these patients is congenital hereditary urea cycle disorders.
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ranking = 6
keywords = cerebral
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8/438. Progressive catatonia.

    We present the case of a young man with a diagnosis of a childhood-onset pervasive developmental disorder who developed a progressive neurologic deterioration with persistent catatonia and right hemiparesis. On his initial evaluation approximately three years after the onset of mutism, he manifested right hemiparesis and catalepsy. Two years later, although catalepsy had subsided, motor function had deteriorated so that he could not use his hands to feed or dress himself. Oral-facialbuccal dyskinesia manifested by blepharospasm and grimacing were present constantly during waking hours. Quantitative electroencephalography demonstrated markedly decreased amplitude, a finding associated with catatonia. Left sural nerve biopsy indicated large axon cylinder degeneration. Left deltoid biopsy demonstrated perimysial fibrosis and type II fiber predominance. Although magnetic resonance imaging of the head without contrast was normal, positron emission tomography indicated hypometabolism of the right cerebral and the right cerebellar hemispheres. The patient continues to deteriorate despite a course of 25 electroconvulsive treatments. He continues to manifest criteria for catatonia including motoric immobility, mutism, and peculiarities of voluntary movement such as prominent grimacing. We suspect an inherited neurodegenerative disorder. Since catatonia is a treatable condition frequently associated with medical and neurological diseases, examination for the features of catatonia must be included in the assessment of patients with progressive brain degeneration. This report is an attempt to clarify the traits of a serious variant of progressive brain degeneration.
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ranking = 1.1166606121474
keywords = cerebral, brain
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9/438. tremor and seizures associated with chronic manganese intoxication.

    tremor and seizures developed in a 2-year-old girl receiving total parenteral nutrition. T1-weighted images on MRI revealed areas of hyperintensity in the basal ganglia, brainstem and cerebellum. blood manganese was elevated. The symptoms and MRI abnormalities disappeared after withdrawal of manganese administration. The recommendation of daily parenteral manganese intake was discussed.
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ranking = 0.058330306073714
keywords = brain
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10/438. Diaschisis in chronic viral encephalitis with Koshevnikov syndrome.

    The authors report a 61-year-old man with chronic viral encephalitis and Koshevnikov syndrome occurring 42 months after initial symptom of right hemiparesis. Serial computed tomography of the brain showed changes in the attenuation of the left temporal lobe lesion over time. Magnetic resonance images of the brain showed enlargement of left temporoparietooccipital lobes with cortical gyral enhancement on T1-weighted images following intravenous administration of gadolinium-DTPA. 99mTc-HMPAO single-photon emission computerized tomography showed increased radioactivity and hyperperfusion in the left temporoparietal region with paradoxically decreased local tissue perfusion at the contralateral right hemisphere. Follow-up magnetic resonance images of the brain 4 years later showed atrophy of bilateral cerebral hemispheres. We postulate that a "transcallosal diaschisis" with subsequent degeneration is a possible mechanism. A brain biopsy from the left temporal lobe lesion showed pictures compatible with viral encephalitis probably herpes simplex encephalitis.
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ranking = 1.2333212242949
keywords = cerebral, brain
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