1/30. 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.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
2/30. 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.- - - - - - - - - - ranking = 0.66666666666667keywords = brain (Clic here for more details about this article) |
3/30. 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.- - - - - - - - - - ranking = 0.33333333333333keywords = brain (Clic here for more details about this article) |
4/30. 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).- - - - - - - - - - ranking = 0.66666666666667keywords = brain (Clic here for more details about this article) |
5/30. 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.- - - - - - - - - - ranking = 0.66666666666667keywords = brain (Clic here for more details about this article) |
6/30. Brain MRI findings in influenza A-associated acute necrotizing encephalopathy of childhood.Acute necrotizing encephalopathy following influenza A is frequently reported from japan and taiwan but is very rarely seen in Western countries. We describe a 10-year-old boy with acute necrotizing encephalopathy, who developed symmetrical thalamic and brain stem lesions seen on magnetic resonance imaging (MRI). Serological confirmation of influenza A was made 2 weeks after the onset of symptoms. The child made a full recovery. This case is interesting because of its rarity in European countries, the striking brain MRI findings and the good neurological outcome.- - - - - - - - - - ranking = 0.66666666666667keywords = brain (Clic here for more details about this article) |
7/30. Two episodes of leukoencephalitis associated with recombinant hepatitis b vaccination in a single patient.Cases of central nervous system demyelination have been reported after recombinant hepatitis b vaccination, but no causal link has been clearly demonstrated. We present the first case report involving the occurrence of 2 episodes of leukoencephalitis in a previously healthy patient after vaccination and rechallenge with hepatitis b vaccine.- - - - - - - - - - ranking = 0.13476003233768keywords = central nervous system, nervous system (Clic here for more details about this article) |
8/30. Post-infectious central and peripheral nervous system diseases complicating mycoplasma pneumoniae infection. Report of three cases and review of the literature.Three patients with a central and peripheral nervous system disease complicating a mycoplasma pneumoniae (M. pn.) infection are presented. Patient 1 suffered from bilateral optic neuritis as well as acute guillain-barre syndrome recovering after plasmapheresis. The two other patients suffered from severe haemorrhagic leukoencephalitis (Hurst) which only could be contained by aggressive decompressive craniectomy with duraplasty. All three illnesses were clearly shown to be associated with M. pn. infection.Our three patients represent the full scale of central nervous (CNS) (cerebral and myelitic) as well as peripheral nervous system (PNS) (GBS, optic neuritis) manifestation of a disease caused by the same pathogenetic - post-infectious - mechanism; pathogenic CNS and PNS epitopes might be shared in post-infectious neurological disease following M. pn. infection.- - - - - - - - - - ranking = 0.30480689068708keywords = nervous system (Clic here for more details about this article) |
9/30. Pediatric acute hemorrhagic leukoencephalitis: report of a surviving patient and review.Acute hemorrhagic leukoencephalitis (AHLE) is a rare, fulminant CNS demyelinating condition usually diagnosed at autopsy. We report the clinical, laboratory, radiographic, and pathologic features of the first nonfatal case of pediatric AHLE confirmed by brain biopsy. Pathologic diagnosis of this condition may be critical to exclude more-common processes and to expedite the decision to administer high-dose corticosteroid therapy, which is potentially lifesaving.- - - - - - - - - - ranking = 0.33333333333333keywords = brain (Clic here for more details about this article) |
10/30. Possible acute hemorrhagic leukoencephalitis manifesting as intracerebral hemorrhage on computed tomography--case report.A 15-year-old girl presented with meningeal irritation and bilateral cerebral signs after contracting influenza. A lumbar puncture revealed bloody cerebrospinal fluid and polymorphonuclear predominant pleocytosis with an elevated protein level and normal glucose level. Computed tomography showed a hematoma in the right basal ganglia and lateral ventricles. Symmetrical low density areas were also noted in the bilateral white matter. The preliminary diagnosis was hemorrhagic cerebrovascular disease of unknown cause. However, her neurological condition deteriorated. Magnetic resonance (MR) imaging showed diffuse high intensity signals in the bilateral white matter and small spotty lesions, indicating hemorrhages in various stages. The final diagnosis was acute hemorrhagic leukoencephalitis (AHL). However, high-dose steroid administration and plasmapheresis failed to improve her condition. hypothermia could not control her intracranial pressure and she died 12 days after admission. The neuroimaging findings indicated the histological characteristics of AHL, but the hematoma formation is rare. AHL is a fulminant form of brain demyelination and can be fatal, so early diagnosis and aggressive treatment are important for successful recovery. Therefore, early investigation by MR imaging is necessary.- - - - - - - - - - ranking = 0.33333333333333keywords = brain (Clic here for more details about this article) |
| | Next -> |