Decreased cerebellar blood flow in postinfectious acute cerebellar ataxia

Nagamitsu, Shinichiro; Matsuishi, Toyojiro; Ishibashi, Masatoshi; Yamashita, Yushiro; Nishimi, Toshihiro; Ichikawa, Koutarou; Yamanishi, Koichi; Kato, Hirohisa

doi:10.1136/jnnp.67.1.109

Cited by 30 publications

(12 citation statements)

References 8 publications

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“…Acute cerebellar ataxia generally shows normal radiological findings on brain CT and MRI. However, a SPECT has demonstrated diminished perfusion of the cerebellum in patients with acute cerebellar ataxia, 9 as shown in our patient.…”

Section: Discussionsupporting

confidence: 72%

Case of acute cerebellar ataxia associated with primary Epstein–Barr virus infection

Harai

Kanegane

Ito

et al. 2010

Pediatrics International

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Key words acute cerebellar ataxia, Epstein-Barr virus, infectious mononucleosis, T cell receptor Vb repertoires.Most primary Epstein-Barr virus (EBV) infections are asymptomatic; however, such infections can cause infectious mononucleosis (IM), which is characterized by a prolonged fever, cervical lymphadenopathy, hepatosplenomegaly, and peripheral lymphocytosis with atypical lymphocytes. IM is sometimes associated with neurological complications, including meningitis, encephalitis, acute demyelinating encephalomyelitis, cranial nerve palsy, cerebellitis, myelitis, and visual seizure with metamorphopsia, that is, "Alice in Wonderland" syndrome. 1,2 Here we describe a case of acute cerebellar ataxia associated with a primary EBV infection and discuss its pathogenesis. Case ReportA 7-year-old boy developed fever and a sore throat and visited a local hospital, where antibiotics (cefditoren pivoxil) were administered. Three days later he had skin eruptions on his hips and thigh. The eruption thereafter expanded over his face and back, but subsided within a few days. Eight days later, he presented with ataxia and a gait disturbance. On the next day, he also had dysphasia and was admitted to Toyama University Hospital. His medical history included bronchial asthma at 3 years of age, Kawasaki disease at 4 years of age and allergic rhinitis at 7 years of age.A physical examination at the time of his admission revealed the patient to be febrile, but his consciousness was alert. A general examination showed bilateral tonsillitis, but no lymphadenopathy or hepatosplenomegaly. A neurological examination revealed the deep tendon reflexes to be diminished and the Babinski reflex was negative. He showed a normal finger-nose test and no tremors, but ataxia in his trunk and a gait disturbance were observed. In addition, a speech disturbance was observed.Laboratory studies showed mild leukocytosis (9470/mL) with 46.5% neutrophils, 2% eosinophils, 4.5% monocytes, 37% lymphocytes, and 9% atypical lymphocytes. Other studies including electrolytes and liver function were normal. No Streptococcus pyogenes or adenovirus were detected with a throat swab culture. Cerebrospinal fluid (CSF) analysis revealed 1/3 leukocytes/mL, a protein of 20.1 mg/dL and a glucose of 61 mg/dL. A serological test for EBV showed viral capsid antigen (VCA)-immunoglobulin (Ig) G of 1 : 1280, VCA-IgM of 1 : 160, early antigen (EA)-IgG < 1 : 10, EA-IgM < 1 : 10, EBV nuclear antigen <1 : 10, thus indicating a primary EBV infection. A polymerase chain reaction (PCR) analysis for EBV was positive (521 copies/mL) in the serum, but negative in the CSF. A lymphocyte subset analysis showed an increased number of human leukocyte antigen-DR-positive (activated) CD3 + T cells (55%) and an inverted CD4/CD8 ratio (9.6%/54% = 0.18), thus suggesting a primary EBV infection.Computed tomography (CT) and magnetic resonance imaging (MRI) of the brain revealed no abnormalities. Brain singlephoton emission computed tomography (SPECT) showed a mild hypoperfusion in the bilateral cere...

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Section: Discussionsupporting

confidence: 72%

Case of acute cerebellar ataxia associated with primary Epstein–Barr virus infection

Harai

Kanegane

Ito

et al. 2010

Pediatrics International

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“…A few authors also reported on perfusion-SPECT in assessing cerebellar inflammation. Nagamitsu et al [27] stress the importance that cerebellar hypoperfusion (demonstrated with SPECT) is a more sensitive marker than MRI in AC. Their findings are based on five proven cases of AC.…”

Section: Discussionmentioning

confidence: 98%

MRI findings in acute cerebellitis

Bruecker¹,

Claus²,

Demaerel³

et al. 2004

Eur Radiol

112

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Acute cerebellitis is an inflammatory process involving the cerebellum. We report the clinical, CT and MRI features of four cases and a review of the literature. Bilateral diffuse hemispheric abnormalities represent the most common imaging presentations. Our observations demonstrate the various imaging appearances of acute cerebellitis. Simultaneous involvement of both hemispheres and the vermis has not been reported previously. The development of cerebellar atrophy following an initial normal MR imaging examination is also a new finding. In atypical clinical presentation, MR imaging can lead to the diagnosis. MR imaging findings have, however, no prognostic value.

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“…Imaging plays a modest role in diagnosis; T2 signal change of the cerebellum is widely documented [60, 61] but structural MRI findings maybe normal if patients are imaged early in the course of the illness [62, 63]. While SPECT perfusion has been reported as a potential diagnostic biomarker in the course of the illness, reports vary with both increased and decreased perfusion demonstrated alongside normal MR appearances of the cerebellum [62, 63]. The pattern of cerebellar involvement is variable, though with the Purkinje cell a frequent immunological target in immune-mediated ataxia of which nature there is often a predominant cerebellar cortical volume as tends to be found in other immune ataxias [59].…”

Section: Section 5: Imaging In Immune-mediated Ataxiasmentioning

confidence: 99%

Consensus Paper: Radiological Biomarkers of Cerebellar Diseases

et al. 2014

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Hereditary and sporadic cerebellar ataxias represent a vast and still growing group of diseases whose diagnosis and differentiation cannot only rely on clinical evaluation. Brain imaging including magnetic resonance (MR) and nuclear medicine techniques allows for characterization of structural and functional abnormalities underlying symptomatic ataxias. These methods thus constitute a potential source of radiological biomarkers, which could be used to identify these diseases and differentiate subgroups of them, and to assess their severity and their evolution. Such biomarkers mainly comprise qualitative and quantitative data obtained from MR including proton spectroscopy, diffusion imaging, tractography, voxel-based morphometry, functional imaging during task execution or in a resting state, and from SPETC and PET with several radiotracers. In the current article, we aim to illustrate briefly some applications of these neuroimaging tools to evaluation of cerebellar disorders such as inherited cerebellar ataxia, fetal developmental malformations, and immune-mediated cerebellar diseases and of neurodegenerative or early-developing diseases, such as dementia and autism in which cerebellar involvement is an emerging feature. Although these radiological biomarkers appear promising and helpful to better understand ataxia-related anatomical and physiological impairments, to date, very few of them have turned out to be specific for a given ataxia with atrophy of the cerebellar system being the main and the most usual alteration being observed. Consequently, much remains to be done to establish sensitivity, specificity, and reproducibility of available MR and nuclear medicine features as diagnostic, progression and surrogate biomarkers in clinical routine.

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Decreased cerebellar blood flow in postinfectious acute cerebellar ataxia

Cited by 30 publications

References 8 publications

Case of acute cerebellar ataxia associated with primary Epstein–Barr virus infection

Case of acute cerebellar ataxia associated with primary Epstein–Barr virus infection

MRI findings in acute cerebellitis

Consensus Paper: Radiological Biomarkers of Cerebellar Diseases

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