Acquired sensorimotor polyneuropathy in an adolescent boy with primary intracranial sarcoma
| Autor: | Jens Schallner, Katharina Sell, Gabriele Hahn, Meinolf Suttorp, Valentin A. Schriever, Martin Smitka, M. von der Hagen, Katja Storch |
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| Rok vydání: | 2019 |
| Předmět: |
Male
Cerebellum Pathology medicine.medical_specialty Ataxia Adolescent Lesion Polyneuropathies 03 medical and health sciences 0302 clinical medicine Cerebrospinal fluid 030225 pediatrics medicine Humans medicine.diagnostic_test Brain Neoplasms business.industry Brain biopsy Sarcoma General Medicine medicine.disease medicine.anatomical_structure Pediatrics Perinatology and Child Health Neuropathic pain Neurology (clinical) Brainstem medicine.symptom business 030217 neurology & neurosurgery Paraneoplastic Syndromes Nervous System |
| Zdroj: | European Journal of Paediatric Neurology. 23:662-667 |
| ISSN: | 1090-3798 |
| DOI: | 10.1016/j.ejpn.2019.04.005 |
| Popis: | Acquired polyneuropathies (PN) are rare in childhood and adolescent. We report on a 15-year-old male patient who presented with progressive gait instability, ataxia, neuropathic pain, distal muscle weakness and progressive loss of ambulation. Nerve conduction studies (NCS) revealed a progressive demyelinating sensorimotor polyneuropathy predominantly of the lower limbs. Cerebrospinal fluid (CSF) analyses revealed a cytoalbuminologic dissociation. Extensive diagnostic workup for autoantibodies and inflammatory markers was inconclusive. Corticosteroids and intravenous immunoglobulins did not affect. Cranial MRI revealed leptomeningeal enhancement of the cerebellum and the brainstem. Brain biopsy of the cerebellar lesions revealed an unclassifiable sarcoma. The patient was treated according to the CWS guidance study resulting in a decrease in enhanced lesion size. After two years NCS still revealed a demyelinating sensorimotor PN. This case report describes for the first time the clinical course of a chronic PN, putative paraneoplastic, associated with isolated unclassifiable CNS-sarcoma in an adolescent patient. Paraneoplastic pathogenesis should be considered in an unusual sequence of subacute progressive neurological symptoms even in children and adolescents. |
| Databáze: | OpenAIRE |
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