1/38. 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 = encephalopathy, brain, cerebral (Clic here for more details about this article) |
2/38. 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 = 1.5985848619169keywords = encephalopathy, brain (Clic here for more details about this article) |
3/38. 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 = 1.1987478861937keywords = encephalopathy, brain, cerebral (Clic here for more details about this article) |
4/38. 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.- - - - - - - - - - ranking = 0.99851819339478keywords = encephalopathy (Clic here for more details about this article) |
5/38. 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 = 0.7997703072011keywords = encephalopathy, brain (Clic here for more details about this article) |
6/38. 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.- - - - - - - - - - ranking = 0.19970363867896keywords = encephalopathy (Clic here for more details about this article) |
7/38. 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 = 1.1992257624363keywords = encephalopathy, brain, cerebral (Clic here for more details about this article) |
8/38. Craniectomy in severe, life-threatening encephalitis: a report on outcome and long-term prognosis of four cases.OBJECTIVE: To report the feasibility of craniectomy with duraplasty in four patients with life-threatening encephalitis and, in particular, their long-term outcome. DESIGN: Report of four cases, analysis of the acute clinical course and neurological long-term sequelae. RESULTS: Generous craniectomy with duraplasty was performed in four patients with life-threatening encephalitis leading to decortication and decerebration. This treatment approach reduced intracranial pressure. The long-term sequelae (1.5-8 years after craniectomy) confirmed its appropriateness, having led to full neurological (cerebral) function, resocialization, and reintegration into their professional life in all four patients. CONCLUSION: Craniectomy with dural augmentation is a treatment approach in cases of severe space-occupying encephalitis, not only saving the patient's life but also leading to favorable long-term outcome.- - - - - - - - - - ranking = 4.8177877301088E-5keywords = cerebral (Clic here for more details about this article) |
9/38. 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 = 1.199177584559keywords = encephalopathy, brain (Clic here for more details about this article) |
10/38. 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 = 4.8177877301088E-5keywords = cerebral (Clic here for more details about this article) |
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