1/51. Acute hemorrhagic encephalitis (Hurst disease) associated with neuroaxonal dystrophy. Two cases that fulfil the clinical and neuropathological criteria of acute hemorrhagic encephalitis are described. Histological examination revealed additionally focal changes in the white matter characteristic for neuroaxonal dystrophy. The differences in the clinical course and morphological picture observed in both cases are discussed. ( info) |
2/51. Necrotizing encephalopathy and macrocephaly with mitochondrial complex I deficiency. A neonate presented in the first weeks after birth with vomiting. He was unresponsive, with hypotonia, macrocephaly, and lactic acidosis. The cranial computed tomographic scan revealed a hypodense brain, with increased brain volume and extensive cerebral edema. He died at 6 weeks of age; postmortem examination revealed necrotizing encephalopathy with marked brain edema, spongiosis, thalamic necrosis, and basal ganglia calcifications. Enzyme studies of the mitochondrial respiratory chain revealed complex I deficiency in both muscle and liver. ( info) |
3/51. Clinical diversity in acute necrotizing encephalopathy. Acute necrotizing encephalopathy is a novel disease entity, proposed by Mizuguchi et al in 1995, that shows a characteristic selective and symmetric involvement of the thalamus, brain stem, and cerebellum. It usually leaves sequelae. The etiology of acute necrotizing encephalopathy is unknown. We describe here six patients aged 6 months to 5 years (four boys and two girls). Four cases were typical, and the patients' cranial computed tomographic scans and magnetic resonance imaging showed irreversible symmetric involvement of the thalamus, brain stem, and cerebellum. Three of the patients died, and one was left with severe sequelae. In the other two patients, who had selective reversible thalamic involvement, the disease was mild; one also showed transient unilateral thalamic involvement. These patients recovered completely. We consider the illness in these two patients to fit the criteria of the mild form of acute necrotizing encephalopathy. We believe that acute necrotizing encephalopathy has some clinical diversity, as is seen in other neurologic disorders, and that a mild form could exist. ( info) |
4/51. Electron microscopic and immunohistochemical verification of diagnosis in two archival cases recognized as encephalitis necroticans acuta (ENA) on routine examinations. Two archival cases diagnosed 20 years ago on routine neuropathological methods as encephalitis Necroticans Acuta (ENA) were investigated in EM and by immunohistochemical methods. The previous diagnosis was confirmed only in one case because herpes simplex virus was found. In the second case the intracellular inclusions visible in ME corresponded to measles virus thus previous diagnosis was changed to SSPE. ( info) |
5/51. Acute necrotising encephalopathy of childhood after exanthema subitum outside japan or taiwan. Acute necrotising encephalopathy of childhood (ANE) is an uncommon disease which predominantly affects infants and young children living in japan and taiwan. A multifocal encephalopathy with symmetrical lesions in the thalamus, tegmentum of the brain stem, cerebral periventricular white matter and cerebellar medulla is characteristic. We present the imaging features in a 4-year-old Japanese boy who had been living in germany for 2 1/2 years before presentation. ( info) |
6/51. Interventricular methotrexate therapy for carcinomatous meningitis due to breast cancer: a case with leukoencephalopathy. A 46-year-old woman presented with paraplegia and severe lumbago. She had had a radical mastectomy for left breast cancer 10 years earlier, and 6 months prior to presentation she completed CMF chemotherapy for treatment of retroperitoneal metastasis. CT and MRI to identify potential causes of the paraplegia and lumbago showed leptomeningeal carcinomatosis due to dissemination from invasive recurrence of the retroperitoneal tumor. An Ommaya reservoir was inserted, and infusion of intrathecal methotrexate (MTX; 5 mg twice weekly) began. Her clinical symptoms improved after receiving 53 mg MTX. However, after receiving 83 mg MTX, the patient became dizzy from leukoencephalopathy. Although administration of prednisolone mostly resolved her symptom, the patient died 9 months after the diagnosis of carcinomatous meningitis. ( info) |
7/51. Acute hemorrhagic leukoencephalitis: report of three cases and review of the literature. attention is directed to the diagnosis of acute hemorrhagic leukoencephalitis and the possibility of its clinical recognition is discussed. Three case histories, two diagnosed at autopsy and one clinically, are presented. The latter was treated with very generous doses of steroids and recovered spectacularly. On omission of steroids, a mild, temporary clinical recurrence occurred. Clinical, clinico-pathologic, and pathologic aspects of the disease are discussed, and etiologic factors mentioned. Some thoughts on the relationship of this disease to experimental allergic encephalomyelitis are mentioned. Differential diagnosis is considered and the use of biopsy for confirmation of diagnosis especially from herpes encephalitis considered. A plea is made for the trial of treatment with large doses of steroids. ( info) |
8/51. Varicella-associated acute necrotizing encephalopathy with a good prognosis. A patient with acute necrotizing encephalophathy (ANE) following varicella infection with a good prognosis is reported. A somatosensory evoked magnetic field (SEF) study using a 37-channel-magnetoencephalography system demonstrated normal latency and strength of the first component (N20m) elicited by median nerve stimulation, despite bilateral symmetrical thalamic lesions on MRI. The normal SEF findings and the good prognosis suggested a reversible breakdown of the blood-brain barrier, and an edematous process as the brain pathology. Furthermore, our results support the idea of distinct generators for the three earliest cortical SEF components (N20m, P30m, N45m). ( info) |
9/51. Acute haemorrhagic leucoencephalitis complicating sepsis. A case of acute haemorrhagic leucoencephalitis presenting as fatal septic encephalopathy is reported. The clinical features of this condition are reviewed and the potential for earlier diagnosis is considered. ( info) |
10/51. Acute necrotizing encephalopathy presenting as a basal ganglia syndrome. Acute necrotizing encephalopathy is a relatively new disease. The characteristic clinical findings are of febrile illness followed by rapid deterioration in mental status and seizures. The hallmark of the disease is multifocal bilateral symmetric lesions affecting the thalamus, hypothalamus, brainstem tegmentum, cerebral white matter, and cerebellum. The etiology is unknown, but immune-mediated mechanism was suggested. We present a 12-year-old previously healthy girl who developed increased sleepiness progressing to stupor and coma. magnetic resonance imaging (MRI) of the brain showed the characteristic findings previously described in acute necrotizing encephalopathy. Her mental status improved dramatically with steroid treatment, and the MRI findings resolved completely within 6 months. Following the acute illness, she developed a complex neuropsychiatric disorder consistent with basal ganglia syndrome. ( info) |