<%server.execute "isdev.asp"%> Histiocytosis-related cerebellar leukoencephalopathy Ekici B, Gümüs S, Özmen M, Devecioglu Ö - J Pediatr Neurosci
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Year : 2011  |  Volume : 6  |  Issue : 2  |  Page : 162-163

Histiocytosis-related cerebellar leukoencephalopathy

1 Department of Pediatric Neurology, Istanbul University, Istanbul, Turkey
2 Department of Pediatrics, Istanbul University, Istanbul, Turkey
3 Department of Pediatric Hematology-Oncology, Istanbul University, Istanbul, Turkey

Date of Web Publication13-Feb-2012

Correspondence Address:
Baris Ekici
Ortaköy Dereboyu cad. Arkeon Sitesi A5 blok D3, Beikta, Istanbul
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1817-1745.92859

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How to cite this article:
Ekici B, Gümüs S, Özmen M, Devecioglu Ö. Histiocytosis-related cerebellar leukoencephalopathy. J Pediatr Neurosci 2011;6:162-3

How to cite this URL:
Ekici B, Gümüs S, Özmen M, Devecioglu Ö. Histiocytosis-related cerebellar leukoencephalopathy. J Pediatr Neurosci [serial online] 2011 [cited 2023 Jan 30];6:162-3. Available from: https://www.pediatricneurosciences.com/text.asp?2011/6/2/162/92859

Dear Sir,

A 7-year-old boy was diagnosed as histiocytosis after a peripheral tissue biopsy when he was 2 years old and completed the course of chemotherapy with vinblastin and prednol. Four years after the diagnosis he presented with subacute onset of truncal ataxia, diplopia, and mild appendicular ataxia especially while reaching for objects. He also exhibited dysarthria and tremulousness. Fundoscopic examination was normal and telangiectasis was not confirmed in the conjunctiva or skin. No neurological sign was noted in the cranial or peripheral nerves. Cranial MRI performed for cerebellar signs, revealed bilateral symmetric lesions on the cerebellar dentate nucleus, middle cerebellar peduncle, and dorsal pons defined as hyperintensities on T2 and FLAIR, which are referred to be neurodegeneration [Figure 1] and [Figure 2]. His ataxia gradually improved during follow-up but lesions remained stable in control MRIs performed 3 and 9 months later.
Figure 1: FLAIR MR sequence, hyperintensities at dentate nucleus and middle cerebellar peduncle

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Figure 2: T2 MR sequence, hyperintensities at posterior pons and middle cerebellar peduncle

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CNS involvement of histiocytosis may vary from space occupying lesions - like granulomas in peripheral tissues - to neurodegenerative changes. [1] Neurodegenerative lesions in histiocytosis are a less frequent and well-known complication than the classical histiocytic granuloma-like lesions and these degenerative lesions do not occupy space, do not exert mass effect, and do not show contrast enhancement. [2] Lesions usually involve cerebellar white matter areas and dentate nuclei symmetrically with basal ganglia and brain stem. [3] Reported pathological examinations revealed focal areas of demyelination with gliosis, loss of neuronal cells in the granular, and Purkinje layers of the cerebellar cortex, with no evidence of typical CD1a+ histiocytic infiltration. Latest studies indicate that neurodegeneration can be caused by a profound inflammatory process, dominated by CD8+ lymphocytes. [4] MRI findings do not correlate with neurologic deterioration. Patients can stay free of neurologic symptoms for years but can present with a spectrum of clinical signs ranging from subtle tremor or mild abnormalities of the reflexes, discrete gait disturbance, dysarthria, dysphagia, and motor spasticity to pronounced ataxia, behavioral disturbances, learning difficulties, or even severe psychiatric disease. [5]

   References Top

1.Grois N, Prayer D, Prosch H, Lassmann H; The CNS LCH Co-operative Group. Neuropathology of CNS disease in Langerhans cell histiocytosis. Brain 2005;128:829-38  Back to cited text no. 1
2.Prosch H, Grois N, Wnorowski M, Steiner M, Prayer D. Long-term MR imaging course of neurodegenerative langerhans cell histiocytosis. AJNR Am J Neuroradiol 2007;28:1022-8.  Back to cited text no. 2
3.Van der Knaap MS, Arts WF, Garbern JY, Hedlund G, Winkler F, Barbosa C, et al. Cerebellar leukoencephalopathy: Most likely histiocytosis-related. Neurology 2008;71:1361-7.  Back to cited text no. 3
4.Grois N, Prayer D, Prosch H, Lassmann H. Neuropathology of CNS disease in Langerhans cell histiocytosis. Brain 2005;128:829-38.  Back to cited text no. 4
5.Grois N, Fahrner B, Arceci RJ, Henter JI, McClain K, Lassmann H, et al. Central nervous system disease in Langerhans cell histiocytosis. J Pediatr 2010;156:873-81.  Back to cited text no. 5


  [Figure 1], [Figure 2]


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