Post-infectious acute cerebellar ataxia in children

Acute cerebellar ataxia is a relatively common neurologic disorder among children. Our aim was to characterize the clinical picture, etiology, and prognosis of acute cerebellar ataxia. The medical records of all children with a diagnosis of acute cerebellar ataxia hospitalized in our center and Hasharon Medical Center from 1990 to 2001 were reviewed. The diagnosis of acute cerebellar ataxia was based on the following criteria: acute onset of ataxia with or without nystagmus; absence of known genetic predisposing factors, such as familial degenerative disorders; and absence of drug intoxication, bacterial meningitis, and metabolic disorders. Thirty-nine children were identified; 54% were male; mean age at presentation was 4.8 +/- 3.8 years. All patients were observed for at least 1 year. A prodromal febrile illness was noted in 74.4%: varicella, 31%; mumps, 20%; nonspecific viral infection, 15.4%; mycoplasma, 5%; Epstein Barr virus, 3%. Latency from the prodromal illness to the onset of ataxia was 8.8 +/- 7.4 days. The most common associated neurologic findings were nystagmus and dysmetria. Full gait recovery took less than 2 weeks on average, and the longest duration of neurologic signs was 24 days (mumps-related). Acute cerebellar ataxia in childhood is a self-limited disease. The recovery was faster than that reported in previous publications and was complete in all children without any neurologic sequelae. Imaging studies are needed only in atypical presentation or if there is no spontaneous improvement after 1 to 2 weeks.     

Intravenous immunoglobulin therapy for acute cerebellar ataxia

A case of acute cerebellar ataxia without any prodromal illness showed cerebellar hypoperfusion on 123 I-iodoamphetamine single photon emission computed tomography. The symptoms did not resolve spontaneously or with methylprednisolone pulse therapy but disappeared rapidly with intravenous immunoglobulin therapy.

Conclusion: Intravenous immunoglobulin therapy is worth considering in acute cerebellar ataxia that does not respond to high-dose steroid therapy.     

Tacrolimus induced subacute cerebellar ataxia.

Tacrolimus is a macrolide immunosuppressant that is widely used in transplant surgery. Both mild and major neurologic side effects have been reported in patients receiving tacrolimus. Reversible posterior leukoencephalopathy syndrome is one of the major tacrolimus induced neurologic side effects. We report a case of a tacrolimus induced isolated lesion of the cerebellum with no supratentorial involvement, presenting as a subacute cerebellar ataxia, which was partially reversible upon discontinuing the tacrolimus.     

Transient cerebellopontine demyelinisation revealed by MRI in acute cerebellar ataxia

An eight year old boy was admitted to our ward with a history of abrupt onset of rapidly progressive gait disorder, nausea, vertigo and vomiting. The clinical as well as the labortory findings suggested the diagnosis of acute cerebellar ataxia. Magnetic resonance imaging (MRI), however, showed marked demyelinisation in the cerebellar region and visual evoked potentials were pathologic. After immunosuppression the patient promptly improved clinically and the lesions depicted by MRI disappeared almost completely.     

Spectrum of centrosome autoantibodies in childhood varicella and post-varicella acute cerebellar ataxia

Background  

Sera from children with post-varicella infections have autoantibodies that react with centrosomes in brain and tissue culture cells. We investigated the sera of children with infections and post-varicella ataxia and related conditions for reactivity to five recombinant centrosome proteins: γγ-enolase, pericentrin, ninein, PCM-1, and Mob1.     

The CSF-protein pattern in acute cerebellar ataxia of childhood and intracranial midline tumours

CSF-protein profiles of 25 children with acute cerebellar ataxia and of 39 children with intracranial midline tumours at diagnosis were examined by quantitative zone electrophoresis in agarose gel. The profiles were compared with those obtained from a control group of 86 cases, and those from 61 patients with aseptic meningitis and 40 children with bacterial meningitis. The data from the latter groups demonstrated the CSF-protein pattern of moderate or severe disturbance of the blood-CSF barrier (B-CSF-B), respectively. The children with acute cerebellar ataxia showed minor signs of a B-CSF-B impairment and no increase of -globulin. These findings point to a slight acute vascular lesion. CSF changes indicative of a moderate-to-severe dysfunction of the B-CSF-B occurred in the majority of the patients with cerebellar astrocytomas, pontine gliomas, tumours around the 3rd ventricle, and medulloblastomas. Therefore acute cerebellar ataxia can be differentiated from intracranial midline tumours in most cases by means of CSF-protein electrophoresis. A striking finding was that 12 out of 14 children with medulloblastomas revealed a marked increase of -globulin. Since in 5 of these cases oligoclonal -globulin could be detected, this finding means local immunoglobulin synthesis within the CNS. The marked increase of -globulin which almost exclusively occurred in association with medullo-blastomas allows their seperation from acute cerebellar ataxia and the other tumour groups. Quantitative agarose gel electrophoresis can be a complementary diagnostic test in children with acute ataxia and suspected of having a CNS infection, or in cases with a negative CT brain scan in which intracranial midline tumour is a likely possibility.     

Genetic aspects of congenital cerebellar ataxia

Congenital cerebellar ataxia comprises a heterogeneous group of birth defects involving brain stem and cerebellum, characterised by ataxia, spasticity, choreoathetoid movements and mental retardation. Three children with congenital cerebellar malformations are described. The presenting features were benign enlargement of head, myoclonic jerks, episodes of hypernoia, nystagmus, ataxia, motor delay and mental retardation. Computerised tomography revealed Dandy-Walker cyst in one case and in two cases cerebellar vermis agenesis was associated with a variable degree of brain stem involvement. Retinal dysplasia and post-axial polydactyly were associated abnormalities in one case as reported in Joubert's syndrome, an autosomal recessive type of familial cerebellar vermis dysgenesis. Genetic factors in the pathogenesis of congenital cerebellar ataxia are reviewed. The relevance of computerised tomography, electroretinography and prenatal diagnosis by fetal ultrasound is discussed. Genetic counselling in congenital cerebellar ataxia presenting in the first year of life is important because of the high risk of recurrence.     

Unusual cerebellar ataxia: "worm wobble" revisited.

We present an unusual case of cerebellar ataxia in a 2 year old girl several days after treatment with piperazine citrate for suspected worm infestation. This is the first reported case of delayed onset neurotoxicity following the therapeutic administration of piperazine in a previously well child.     

Acute cerebellar ataxia with human parvovirus B19 infection.

A 2 year old boy developed acute cerebellar ataxia in association with erythema infectiosum. During the disease, genomic DNA and antibodies against human parvovirus B19 were detected in serum but not in cerebrospinal fluid. Parvovirus B19 associated acute cerebellar ataxia might occur due to transient vascular reaction in the cerebellum during infection.     

小儿急性小脑性共济失调11例临床分析

急性小脑性共济失调 (ａｃｕｔｅｃｅｒｅｂｅｌｌａｒａｔａｘｉａ)是小儿特有的综合征[1] ,常发生于各种急性感染之后 ,一般症状和体征只限于小脑功能障碍 ,需与其它较严重的疾病相鉴别。现将近年我们诊治的 11例小儿急性小脑性共济失调分析报告如下。1　临床资料1.1　一般资料男 4例 ,女 7例。年龄 1～ 4岁 5例 ,4岁～ 4例 ,7岁～ 2例。起病时间 1～ 15ｄ。病前有感冒史4例 ,腹泻史 2例。1.2　临床表现步态不稳 11例 ,口齿不清 4例 ,头昏 3例 ,恶心、呕吐 2例 ,不能独坐、竖头 1例。指鼻试验阳性8例 ,跟膝胫试验阳性 7例 ,膝腱反射减低 5…