Acute necrotizing encephalopathy: combined therapy and favorable outcome in a new case

BACKGROUND: Acute necrotizing encephalopathy (ANE) is a rare disease characterized by multiple, symmetrical brain lesions, affecting thalami, brainstem tegmentum, and cerebellar medulla; more inconstantly, other structures are involved, i.e., internal capsules, posterolateral putamen, and deep periventricular white matter. FEATURES: The clinical picture consists of rapidly deteriorating acute monophasic encephalopathy preceded by prodromal febrile illness; the symptoms include hyperpyrexia, convulsions, recurrent vomiting, and coma within 24 h. PROGNOSIS: The outcome is usually poor and approximately 70% of the patients die within a few days from the onset of fever. There is no specific therapy for ANE but, in some patients, the clinical status improved with steroid treatment.     

Familial mitochondrial encephalopathy. A clinicopathologic study]

We report the cases of 2 siblings with progressive encephalopathy. The first symptoms were noted when they were 6 years old. The full clinical picture included myoclonus, seizures, cerebellar ataxia, blindness due to optic atrophy and retinal degeneration, deafness, swallowing difficulties with relatively spared intellectual functions. The course was progressive and led to death within 8 years. The pathological findings included bilateral and almost symmetrical lesions involving the thalami, the colliculi, and the pontine and medullar tegmentum, similar to the changes described in Leigh disease. Neuronal loss and gliosis were noted in the dentate nucleus and in the inferior olive, as in MERRF syndrome. Laminar necrosis of the cerebral cortex could have been due to episodes of severe hypotension before death. Cytochrome c oxidase deficiency was found in case 2. The enzyme deficiency was present in muscle and in fibroblasts in culture.     

A case of transient hypodensity of the caudatum and putamen

Bilateral, approximately symmetrical, hypodense areas located in the caudatum and putamen were seen on CT in a 7-month-old female. These lesions completely disappeared within four weeks in parallel with her clinical signs of muscle hypotonia. Although signs of inflammation were not detected on examination of the blood and cerebrospinal fluid, the etiology of these hypodense lesions was suspected to be a localized viral infection.     

Three cases of central nervous system complications associated with Mycoplasma pneumoniae

We report three new cases of acute central nervous system disease occurring shortly after Mycoplasma pneumoniae infection. The clinical phenotypes were characterized by encephalopathy (n = 2), optic neuritis (n = 1), transverse myelitis (n = 1), and seizures (n = 1). Although there was strong supportive evidence of preceding M. pneumoniae infection, cerebrospinal fluid polymerase chain reaction for M. pneumoniae was negative in all three patients. We propose that these cases resulted from a para-infectious immune-mediated process rather than parenchymal invasion by the microorganism. The two patients treated with steroids improved rapidly, and all three patients have made a full recovery. We review the literature regarding M. pneumoniae central nervous system complications and discuss the proposed pathologic mechanisms; para-infectious immune-mediated disease and parenchymal invasion of the central nervous system. Systematic investigation to discriminate between these two processes will be essential to select appropriate antibiotic and immunomodulatory therapies.     

Acute disseminated encephalomyelitis presenting with bilateral transient amaurosis

A 4-year-old male presented with acute disseminated encephalomyelitis with seizures and transient amaurosis after initial symptoms of a flulike febrile infection 1 week earlier. Immunoglobulin M type antibody titers against Mycoplasma pneumoniae were significantly increased in serum and cerebrospinal fluid. The patient improved appreciably on immunosuppressive therapy with immunoglobulin. This patient exemplifies a rare case of acute disseminated encephalomyelitis presenting with bilateral transient amaurosis as a complication of M. pneumoniae infection. Aydin A, Atasever S, Cakmakci H. Acute disseminated encephalomyelitis presenting with bilateral transient amaurosis.     

生物素-硫胺素反应性基底节病的临床特点(附2例报告)

目的 探讨生物素-硫胺素反应性基底节病(BTRBGD)的临床特点及预后,提高对BTRBGD的认识.方法 回顾性分析2例BTRBGD患儿的临床资料,总结其临床特征并进行文献复习.结果 本组2例患儿为同胞姐弟,例1起病早,有喂养困难、运动发育落后、肌张力障碍、痫性发作等表现,例2仅以头痛为主要表现.2例患儿头颅影像学均提示...     

Opsoclonus secondary to encephalopolyradiculoneuritis due to Mycoplasma pneumoniae

Opsoclonus is characterized by sudden onset of arrhythmic, multidirectional chaotic eye movements, generally associated with a parainfectious or paraneoplasic condition. Opsoclonus is a rare manifestation with no known link with infection by Mycoplasma pneumoniae. We present a case of a 23 year old male patient who presented fever, myalgia, vomiting, respiratory failure, opsoclonus and symmetric flaccid tetraparesis with important lower limb muscle atrophy. Serology and seroreversion for M. pneumoniae were both positive. This case illustrates the possibility that M. pneumoniae may produce a predominantly neurological disease and the need to include this infection in the differential diagnosis of opsoclonus, or polyradiculoneuritis.     

Restless legs syndrome: association with streptococcal or mycoplasma infection

Group A beta-hemolytic streptococcal infections have been reported to cause neuropsychiatric symptoms, such as chorea, tics, and obsessive-compulsive disorder, presumably through autoimmune damage to basal ganglia. Mycoplasma pneumoniae infections have also been reported to cause damage to the basal ganglia. Restless legs syndrome is a movement disorder with focal restlessness, an irresistible desire to move, and exacerbation by long periods of sitting or lying. We present three children with transient restless legs syndrome-like symptoms possibly associated with group A beta-hemolytic streptococcal infection or Mycoplasma pneumoniae infection. One of three patients had persistently elevated enzyme-linked immunosorbent optical density values against human caudate and putamen.     

Multiple system degeneration involving thalamus,reticular formation,pallido-nigral,pallido-Luysian and dentato-rubral systems

Summary An autopsy case of multiple system degeneration is characterized by the following; (1) progressive dementia and abnormal sleep patterns, followed by Parkinsonian symptoms with terminal akinetic mutism: (2) severe symmetrical degeneration in the thalamus, particularly nucl. medialis thalami, the reticular formation of the brain stem, also the pallidonigral, pallido-Luysian and dentato-rubral systems. As far as we know, there is no case in the literature, of combined system degeneration, which shows such a wide anatomical range of lesions as the present one. Clinico-pathological correlation between dementia and degeneration of the thalamus, and between abnormal sleep-consciousness mechanism and degeneration of the reticular formation are discussed. The extrapyramidal symptoms are discussed from the clinico-pathological aspect. In addition, peculiar eosinophilic bodies are described, which were most frequently found in the putamen.     

胼胝体变性的临床特点与影像学特征研究

目的　研究分析了一组胼胝体变性 (MBD)患者的临床表现和影像学特征 ,探讨不同临床分型的特点与诊断方法。方法　 4例长期酗酒男性MBD患者 ,急性型 1例 ,慢性型 3例 ,均经CT检查 ,3例经头MR检查 ,1例行尸检病理检查。结果　急性型主要表现为意识障碍、精神症状与癫 ;慢性型以痴呆、精神症状为主要表现 ;CT与MR可见胼胝体膝部和压部病灶。CT病灶为低密度 ,MR表现为T1WI低信号 ;T2 WI高信号 ;2例伴发双侧大脑半球白质对称性病灶 ,1例出现脑干对称性病灶。尸检病理提示 :胼胝体呈多灶性脱髓鞘与坏死改变。结论　急性型MBD意识障碍突出 ,病程进展迅速 ;慢性型以痴呆、精神症状和锥体束损害为主要表现 ;CT与MR为其生前诊断的主要手段 ,以胼胝体膝部、压部对称性病灶为其主要异常表现。     

A case of acute encephalopathy with biphasic seizures and late reduced diffusion associated with Streptococcus pneumoniae meningoencephalitis

Acute encephalopathy with biphasic seizures and reduced diffusion (AESD) encompasses a group of encephalopathy characterized by biphasic seizures and disturbance of consciousness in the acute stage followed in the subacute stage by reduced diffusion in the subcortical white matter on magnetic resonance imaging. The etiology of AESD is viral infection and associated pathological changes. Here we report the first case of AESD caused by bacterial infection (Streptococcus pneumoniae meningitis) in a 1-year-old boy.     

Gliomatosis Cerebri Presenting as a Parkinsonian Syndrome

We report a 70-year-old man who was hospitalized after a left-sided partial seizure progressed to grand mal seizures. Three years before, the patient had presented with the main symptoms of bradykinesia and gait disturbance and was diagnosed with parkinsonism resistant to L-dopa. At the latest admission, extensive diffuse white matter high-intensity areas were present on T2-weighted magnetic resonance imaging images, and stereotactic brain biopsy showed that these lesions were gliomatosis cerebri (GC). This is the first reported case in which extensive lesions in the bilateral white matter have been associated with parkinsonism as the main clinical feature; only one other case has been reported in which GC presented as parkinsonism, and this differed from the current case with respect to localization and symptoms. We speculate that damage to the thalamocortical projections and functional impairment due to demyelination could have caused parkinsonism in our patient, and we discuss the differential diagnosis.     

Dysembryoplastic neuroepithelial tumour as a cause of coma

Dysembryoplastic neuroepithelial tumour (DNT), a non-evolutive intracranial cortical lesion, is generally associated with epileptic seizures principally among youths. A case of a DNT which presented with uncommon clinical features, characterized by severe intracranial hypertension and progressive blindness warranting emergency surgery, is documented. In addition to the classical radiological and neuropathological features characteristic of DNTs there was a large haemorrhagic cystic haematoma as a result of repeated dissections and/or ruptures of the abnormal vessels in areas, explaining some of the atypical clinical symptoms. Therefore the need for a regular, careful clinical and radiological follow-up of cases with cystic DNTs is strongly recommended.     

Cerebral microhemorrhage in Marchiafava–Bignami disease detected by susceptibility-weighted imaging

Marchiafava–Bignami disease (MBD) is a rare alcohol-associated disorder. Clinical features include not only disturbed consciousness, dysarthria, tetraparesis, astasia-abasia, and symptoms of interhemispheric disconnection as initial symptoms but also cognitive deficits as clinical outcomes. The clinical significance of cerebral microhemorrhage (CMH) has been recognized in patients with cognitive deficits; however, the presence of CMH in patients with MBD has not been emphasized. The aim of the present study was to clarify the relationship between CMH and MBD. For this purpose, we report four patients with MBD, who showed asymmetrical hypointense areas in multiple cortico-subcortical regions on susceptibility-weighted imaging (SWI). All cases had a history of chronic alcohol abuse and symmetrical lesions in the entire corpus callosum. These patients’ clinical symptoms included not only coma, dysarthria, and astasia-abasia as initial symptoms but also dementia as a clinical outcome. SWI showed asymmetrical hypointense areas in the multiple cortico-subcortical regions, indicating the presence of CMH. Compared with patients with normal cognitive function, demented patients showed higher severity of CMH. Our report would indicate that CMH is an important factor indicating the severity of dementia in patients with MBD.     

Bickerstaff brainstem encephalitis associated with Mycoplasma pneumoniae infection

Bickerstaff brainstem encephalitis is a clinical syndrome of ophthalmoplegia, cerebellar ataxia, and central nervous system signs and is associated with the presence of anti-GQ1b antibodies. There is a clinical continuum between Bickerstaff brainstem encephalitis and Miller Fisher syndrome. We describe the case of an 11-year-old boy with encephalopathy, external ophthalmoplegia, brainstem signs, and ataxia with raised titers of anti-GQ1b antibodies. He presented following a respiratory illness and had laboratory evidence of recent infection with Mycoplasma pneumoniae. M pneumoniae infection has been associated with both Bickerstaff brainstem encephalitis and Miller Fisher syndrome. This is only the second case in the literature of Bickerstaff brainstem encephalitis with raised titers of anti-GQ1b antibodies described in association with M pneumoniae infection. The patient responded to intravenous immunoglobulin administration.     

Can meningoencephalitis be caused by Chlamydiae pneumoniae?

INTRODUCTION: Meningoencephalitis attributed to Chlamydiae pneumoniae has rarely been reported in the literature. OBSERVATION: We describe the case of a 21-year-old-man admitted for a meningeal syndrome and fever associated with respiratory symptoms and a temporal syndrome. Even though the clinical presentation and certain serological results suggested Chlamydiae infection, some of the biological data raised the possibility of Epstein Barr Virus infection. Intravenous fluoroquinolone therapy led to a successful outcome. CONCLUSION: Meningoencephalitis associated with non neurological symptoms, especially respiratory symptoms, is suggestive of Chlamydia pneumoniae infection. A broad spectrum antibiotic should be prescribed.     

Posterior cerebral artery occlusion associated with Mycoplasma pneumoniae infection

Cerebral infarction is a rare complication of Mycoplasma pneumoniae infection. In all cases previously reported in the literature, vascular occlusion occurred in the anterior brain circulation, either the internal carotid or the middle cerebral artery. We report a case of a child with posterior cerebral artery occlusion and resultant hemiparesis associated with M. pneumoniae infection.     

Clinical, neuroimaging and electroencephalographic findings of encephalopathy occuring after the ingestion of "sugihiratake" (Pleurocybella porrigens), an autumn mashroom: a report of two cases]

We report a 54-year-old man (case 1) and a 79-year-old woman (case 2) who presented with encephalopathy of unknown cause. Both patients were on hemodyalysis and took an autumn mashroom, "sugihiratake" (Pleurocybella porrigens), two to three weeks prior to the onset of neurological alterations. The clinical syndrome of those patients was characterized by weakness and involuntary movements of the extremities (cases 1 and 2) or dysarthria (case 1) at the onset of the disease and subsequent intractable focal motor seizures, resulting in generalized status epilepticus or comatose state, six (case 1) or three (case 2) days after the disease onset. Epileptic seizures were gradually improved in both cases. On brain MRI of case 1, no relevant lesions were detectable at the onset day, but, 6 days after onset, T2-high intensity lesions were noted in the subcortical white matter of the insular cortex, claustrum, external capsule, putamen and globus pallidus on both sides. On brain CT scan of case 2, there were no apparent lesions at the onset day, but, 4 days after onset, low density areas were noted bilaterally in the subcortical white matter of the insular cortex. Electroencephalography of the two patients taken on a day of comatose state showed periodic synchronous discharge (PSD), which disappeared when their consciousness levels were improved. As far as we have examined, there was no findings to suggest the cause of the encephalopathy in routine laboratory examinations and various viral antibody studies of the blood and cerebrospinal fluid. The reported patients could constitute a newly recognized disease entity, "sugihiratake" encephalopathy. Our observations suggest that it can be an encephalopathy with subacute progression and affect mainly the basal ganglia. Neuroimaging study and electroencephalographic findings may help the diagnosis, although they may remain unremarkable for several days after onset of the neurological alterations.     

Acute necrotizing encephalopathy during novel influenza A (H1N1) virus infection

A novel swine‐origin influenza A (H1N1) virus was recently identified in Mexico. Some cases of infection with neurological complications have been reported to date. We report a case of acute necrotizing encephalopathy associated with the novel H1N1 virus in a 2‐year‐old European girl who suddenly developed fever, seizures, and altered mental status. Brain and spinal cord magnetic resonance imaging showed bilateral symmetrical lesions of the insulae, thalami, geniculate bodies, and pons tegmentum suggestive of an acute necrotizing encephalopathy. An involvement of meninges and spinal cord was observed configuring an acute necrotizing meningoencephalomyelitis. ANN NEUROL 2010;68:111–114