Accepted Manuscript A case of acute cerebellitis with a unique sequential change on MRI Saeko Suzuki , M.D Akimune Kaga , M.D., Ph.D Natsuko Kusaka , M.D Mitsugu Uematsu , M.D., Ph.D Kazuhiro Haginoya , M.D., Ph.D Yu Katata , M.D Yoko Matsubara , M.D Tomoyuki Ishida , M.D Setsuko Kitaoka , M.D., Ph.D Satoru Kumaki , M.D., Ph.D PII:
S0887-8994(14)00278-1
DOI:
10.1016/j.pediatrneurol.2014.05.002
Reference:
PNU 8356
To appear in:
Pediatric Neurology
Received Date: 10 April 2014 Revised Date:
26 April 2014
Accepted Date: 4 May 2014
Please cite this article as: Suzuki S, Kaga A, Kusaka N, Uematsu M, Haginoya K, Katata Y, Matsubara Y, Ishida T, Kitaoka S, Kumaki S, A case of acute cerebellitis with a unique sequential change on MRI, Pediatric Neurology (2014), doi: 10.1016/j.pediatrneurol.2014.05.002. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
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ACCEPTED MANUSCRIPT Case report
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A case of acute cerebellitis with a unique sequential change on MRI.
Saeko Suzuki M.D.a, Akimune Kaga M.D., Ph.D.a, Natsuko Kusaka M.D.a, Mitsugu Uematsu M.D., Ph.D.b, Kazuhiro Haginoya M.D., Ph.D.c, Yu Katata M.D.a Tomoyuki Ishida M.D.a
Setsuko Kitaoka M.D., Ph.D.a, Satoru
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Matsubara M.D.a
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Kumaki M.D., Ph.D.a, d
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Yoko
Department of Pediatrics, Sendai Medical Center, bDepartment of Pediatrics, Tohoku
University School of Medicine,
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Department of Pediatrics Neurology, Takuto
Rehabilitation Center for Children, Sendai, Miyagi, Japan.
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Corresponding author: Satoru Kumaki, Department of Pediatrics, Sendai Medical
Center, 2-8-8 Miyagino, Miyagino-ku, Sendai, Miyagi 983-8520, Japan. E-mail:
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[email protected], tel: +81-22-293-1111, fax: +81-22-291-8114.
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Running title: Unique MRI finding in acute cerebellitis
Manuscript word count: 910 words.
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ACCEPTED MANUSCRIPT Abstract
BACKGROUND: Acute cerebellitis with unilateral onset is extremely rare, and MRI is a useful method for demonstrating cerebellar involvements. PATIENT: We report a 12
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year-old girl with acute cerebellitis with a unique sequential change on her MRI. RESULTS: The patient’s brain MRI first showed cortical lesions mainly in the right cerebellar hemisphere. These subsequently disappeared, and at the same time, new
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lesions appeared in the opposite cerebellar hemisphere. All the lesions were confined to gray matter in the cerebellum, and were isotense on diffusion weighted imaging (DWI)
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and high intense on the apparent diffusion coefficient (ADC) map, consistent with the characteristic of vasogenic edema. CONCLUSION: The sequential MRI demonstrates conversion of hemicerebellitis from unilateral to bilateral cerebellitis during subacute phase, and vasogenic edema might be contributing to the pathogenesis of acute
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cerebellitis in this patient.
Key words: cerebellitis, unilateral onset, magnetic resonance imaging, diffusion
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Introduction
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weighted imaging, apparent diffusion coefficient map.
Acute cerebellitis is a disorder characterized by sudden onset of cerebellar dysfunction resulting from para-infectious, postinfectious, or postvaccination cerebellar inflammation [1]. Because children with acute cerebellitis may not present with typical cerebellar signs, neuroimaging including the MRI is the most useful method for demonstrating cerebellar involvement. The characteristic MRI findings are diffuse bilateral cortical swelling and high intensity of the cerebellum on T2-weighted images (T2-WI) [2]. However, unilateral onset is exceedingly rare, and there is very little data
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on diffusion weighted imaging (DWI) and the apparent diffusion coefficient (ADC) map [3]. Here, we report unique brain MRI findings with unilateral lesions at onset in a
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12-year-old girl with acute cerebellitis.
Case report
A 12-year-old girl with an unremarkable medical history was admitted to our hospital
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because of occipital headaches and low-grade fever for five days followed by vomiting, vertigo, and diplopia for one day. On admission, her consciousness was clear but her
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speech was slurred. There were diplopia and dysarthria, but the remainder of her neurological exam was normal. Her vital signs and laboratory data including levels of serum ammonia, glucose, creatine kinase and amylase were normal. The blood cell counts showed leukocytes 5100/mm3 with 70.5% neutrophils, 21.9% lymphocytes and 3.5% monocytes, and platelets 212,000/mm3. Cerebrospinal fluid (CSF) analysis
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showed 917 leukocytes/mm3 (65% mononuclear cells and 35% polymorphonuclear cells), glucose 47 mg/dl and protein 66 mg/dl. Myelin basic protein and oligoclonal
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immunoglobulin G in CSF were negative. The MRI of the brain revealed partial edema, evident as hyperintensity in the cortical lesions of the right base of the cerebellar
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hemisphere, with minimal involvement of the left hemisphere, based on T2-WI (Fig. 1A, B). In addition, the lesions were isotense on DWI and high intense on the ADC map (Fig. 1C, D). There were no other intracranial and cervical spine lesions on the MRI. Under a diagnosis of acute cerebellitis and meningitis, the patient was empirically treated with acyclovir, meropenem and minocycline. She also received intravenous dexamethasone 0.6mg/kg/day for three days. After initiation of the therapy, she did not complain of a headache or vertigo. A follow-up CSF analysis on day 12 was normal except for showing 46 leukocytes/mm3. An electroencephalogram taken on day 14 of
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illness showed no abnormal findings. Routine bacterial and viral cultures of CSF were negative. Polymerase chain reaction (PCR) for herpes simplex virus, enteroviruses and Mycoplasma pneumoniae (M. pneumoniae) in the CSF were all negative. Serum IgG
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antibodies against Epstein-Barr virus, mumps virus, cytomegalovirus, rubella virus, and varicella zoster virus were positive, while the IgM antibodies were all negative. In contrast, IgM antibodies against M. pneumoniae were positive, and M. pneumoniae
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particle agglutination (PA) antibody [4] and cold agglutination titers were high (1:320 and 1:64, respectively), although M. pneumoniae PA titer in CSF was low (less than
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1:40).
The patient was discharged from our hospital with no neurological sequelae on day 19 of illness. The initial lesions disappeared from the MRI performed on day 38. However, new lesions appeared in the opposite side of cerebellar hemisphere based on T2-WI (Fig. 1E, F). The lesions were again isotense on DWI and high intense on the
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ADC map (Fig. 1G, H). A follow-up MRI performed on day 69 showed no abnormality except for the minimal residual hyperintensity in the right cerebellar hemisphere on
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Discussion
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T2-WI.
Acute cerebellitis has been reported in association with various infections [1, 2, 5]. In our case, IgM antibodies against M. pneumoniae were positive, and M. pneumoniae PA antibody and cold agglutination titers were high, although M. pneumoniae PA titer in CSF was low. A previous report suggested an autoimmune pathogenesis by demonstrating anti-ganglioside antibodies in the serum in the absence of M. pneumoniae genome in CSF [6]. However, it is difficult to establish a causal relationship between M. pneumoniae infection and acute cerebellitis because the
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ACCEPTED MANUSCRIPT organism is difficult to culture, may co-infect with other viruses and can persist in the throat for weeks as previously described [7].
Acute cerebellitis is an inflammatory process involving the cerebellum, and MRI is a
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useful method for demonstrating cerebellar involvements. The typical MRI appearance of acute cerebellitis has been described previously as cerebellar hemispheric cortical edema with hyperintensity on T2-WI [2, 8]. Lesions in our case were first located
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mainly in the cortical area of the right base of cerebellar hemisphere, which resolved by day 38, and at the same time new lesions appeared in the cortical area of the left base of
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cerebellar hemisphere on T2-WI. Because the lesions were confined to gray matter of the cerebellum, a diagnosis of acute cerebellitis was made. MRI findings of our patient are unique because hemicerebellitis eventually converted to bilateral cerebellitis during subacute phase. The lesions showed normal DWI signal intensities with hyperintensity on the ADC map, which was consistent with a previous report [9]. Pathological high
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signal intensities on ADC map suggest the presence of vasogenic edema without the T2 shining-through effects that observed even on DWI sequences [10]. Of note, one report
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described increased DWI with corresponding low ADC, in keeping with cytotoxic edema, in a patient with acute respiratory syncytial virus cerebellitis [8]. Accumulation
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of information on DWI and ADC maps of acute cerebellitis will be needed to understand the underlying pathological mechanism of acute cerebellitis. Several studies have described cerebellar atrophy on MRI images in some patients with cerebellitis in the convalescent stage [1, 2]. Although the patient has shown no signs of neurological sequelae, developmental delay or reduced intelligence, careful follow-up is necessary.
References
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ACCEPTED MANUSCRIPT encephalomyelitis: report of two cases of brain stem encephalitis. J Neurol
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ACCEPTED MANUSCRIPT Figure legends Figure 1.
MRI images of the brain, performed on admission (A-D, L), and on day 38 (E-H) of
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illness. Brain MRI on admission showed hyperintense lesions mainly in the cortex of the right base of the cerebellar hemisphere, with minimal involvement of the left hemisphere, based on T2-WI (3800/100, TR/TE) axial (A) and coronal (B) images.
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DWI (2600/90, TR/TE, b value: 1000s/mm2) showed normal signal intensities (C), and increased ADC map (D), compatible with vasogenic edema. Subsequent MRI on day 38
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of illness showed new hyperintense lesions in the cortex of the left base of cerebellar hemisphere, although the initial lesions disappeared, based on T2-WI (3800/100, TR/TE) axial (E), coronal (F) images. DWI (3500/90, TR/TE, b value: 1000s/mm2) again showed normal signal intensities (G), and increased ADC map (H).
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are indicated by white arrows.
The lesions
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