Cerebellar Ataxia in Pediatric Patients With Langerhans Cell Histiocytosis

Imashuku, Shinsaku; Ishida, Shuichi; Koike, Kenichi; Ichikawa, Motoki; Minato, Toshinori; Suzuki, Yuki; Kobayashi, Ryōji; Morimoto, Akira

doi:10.1097/00043426-200411000-00009

Cited by 19 publications

(11 citation statements)

References 19 publications

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“…However, it is possible that these incidences may become higher if the follow-up period of our series is much longer than the current median 5 years. In terms of ND disease, our previous analysis of patients with LCH-induced ND disease revealed that only half had CDI [19]. Thus, the six ND disease cases that were detected here in association with CDI do not account for all neurologically impaired cases in the cohorts of the JLSG-96/-02 studies [20].…”

Section: Discussionmentioning

confidence: 56%

Analysis of 43 cases of Langerhans cell histiocytosis (LCH)-induced central diabetes insipidus registered in the JLSG-96 and JLSG-02 studies in Japan

et al. 2011

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To determine the ability of recent systemic chemotherapy protocols to reduce the incidence of central diabetes insipidus (CDI) in Langerhans cell histiocytosis (LCH), 43 CDI cases that belonged to a cohort of 348 pediatric patients with multi-focal LCH who were treated with the JLSG-96/-02 protocols were analyzed. The overall incidence of CDI was 12.4%, but in 24 cases CDI was already present at the time LCH was diagnosed. Thus, CDI developed during or after systemic chemotherapy over a follow-up period of 5.0 (0.2-14.7) years in only 19 patients (5.9%), with 7.4% at 5-year cumulative risk by Kaplan-Meier analysis. In two cases, complete resolution of CDI was noted. Anterior pituitary hormone deficiency was detected in 13 cases, while CDI-associated neurodegenerative disease was observed in six cases. The JLSG-96/-02 protocol appears to effectively reduce the occurrence of CDI. However, novel therapeutic measures are required to reverse pre-existing CDI and to prevent CDI-associated neurological complications.

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Section: Discussionmentioning

confidence: 56%

Analysis of 43 cases of Langerhans cell histiocytosis (LCH)-induced central diabetes insipidus registered in the JLSG-96 and JLSG-02 studies in Japan

et al. 2011

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“…Degenerative CNS disease developed in 3 patients, including 1 patient in the SS‐m group and 2 patients in the MS group. Details on these patients were published previously 14…”

Section: Resultsmentioning

confidence: 99%

Improved outcome in the treatment of pediatric multifocal Langerhans cell histiocytosis

Morimoto

Ikushima

Kinugawa

et al. 2006

Cancer

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BACKGROUND. The treatment outcome of multifocal childhood Langerhans cell histiocytosis (LCH) has not been satisfactory and has resulted in poor therapeutic responses with high mortality and a high incidence of reactivation with late sequelae. To overcome these issues, the Japan LCH Study Group‐96 (JLSG‐96) protocol was conducted prospectively from 1996 to 2001 in Japan. METHODS. Newly diagnosed children with multifocal LCH were classified into 2 groups: a single‐system multisite (SS‐m) group and a multisystem (MS) group. All patients initially were treated on Protocol A, which consisted of 6 weeks of induction therapy with combined cytosine arabinoside, vincristine (VCR), and prednisolone (PSL) followed by 6 months of maintenance therapy. Patients who had a poor response to the induction of Protocol A were switched to a salvage regimen (Protocol B), which consisted of an intensive combination of doxorubicin, cyclophosphamide, VCR, and PSL. RESULTS. In total, 91 patients were treated, including 32 patients in the SS‐m group and 59 patients in the MS group. At the median 5‐year follow‐up, 96.9% of patients in the SS‐m group and 78.0% of patients in the MS group had good response status. Diabetes insipidus developed in 3.1% of patients in the SS‐m group and in 8.9% of patients in the MS group. The overall survival rate at 5 years for the SS‐m and MS groups was 100% and 94.4% ± 3.2%, respectively. CONCLUSIONS. The JLSG‐96 protocol attained very low mortality for pediatric patients with multifocal LCH. Cancer 2006. © 2006 American Cancer Society.

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“…LCH patients presenting with cerebellar ataxia have been rarely reported, and even then exclusively pediatric cases [8, 9, 10, 11, 12]. In those patients, cerebellar ataxia appeared with LCH progression after initial presentation of a skin or bone lesion.…”

Section: Discussionmentioning

confidence: 99%

Late-Onset Langerhans Cell Histiocytosis with Cerebellar Ataxia as an Initial Symptom

et al. 2016

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Late-onset progressive cerebellar ataxia is a diagnostic challenge because of a poor correlation between genotype and phenotype, and a broad range of secondary causes that extend beyond the neurological field. We report the case of a 45-year-old woman admitted after 2 years of slowly progressing cerebellar ataxia, dysarthria, and emotional instability. Notably, she was diagnosed with diabetes insipidus at the age of 35. As ‘idiopathic cerebellar ataxia’ was suspected, diagnostic tests, including genetic testing as well as serum and cerebrospinal fluid analyses, and brain magnetic resonance imaging (MRI) were performed. All results were normal except those of MRI, performed 9 months prior to admission, which showed multiple dot-like white matter lesions with unclear cause. On a repeated brain MRI, a new lesion presenting as a 1.5-cm-sized highly enhancing mass attached to the right frontal skull was found. A sharply marginated lytic skull defect was also evident on skull X-ray, which corresponded to the lesion mass. Given these new radiological findings, a systemic review of the patient’s medical history for rare secondary causes of cerebellar ataxia was performed, with particular attention to her past ‘diabetes insipidus’. The mass, lytic lesion of the skull, white matter lesion, diabetes insipidus, and cerebellar ataxia all suggested a final diagnosis of Langerhans cell histiocytosis (LCH), which was confirmed histopathologically. This is a rare case of late-onset LCH with an unusual initial symptom which underlines the importance of carefully reviewing the patient’s medical history and broadening the search for etiologies beyond the nervous system.

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Cerebellar Ataxia in Pediatric Patients With Langerhans Cell Histiocytosis

Cited by 19 publications

References 19 publications

Analysis of 43 cases of Langerhans cell histiocytosis (LCH)-induced central diabetes insipidus registered in the JLSG-96 and JLSG-02 studies in Japan

Analysis of 43 cases of Langerhans cell histiocytosis (LCH)-induced central diabetes insipidus registered in the JLSG-96 and JLSG-02 studies in Japan

Improved outcome in the treatment of pediatric multifocal Langerhans cell histiocytosis

Late-Onset Langerhans Cell Histiocytosis with Cerebellar Ataxia as an Initial Symptom

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