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Neurological manifestations of Hunter syndrome

L.G. Gutiérrez-Solana [REV NEUROL 2008;47 (Supl. 2):S9-S13] PMID: 19021118 DOI: https://doi.org/10.33588/rn.47S02.2008538

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Volumen 47 | Number S02 | Nº of views of the article 7.269 | Nº of PDF downloads 1.394 | Article publication date 10/11/2008

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ABSTRACT

INTRODUCTION Mucopolysaccharidosis type II, or Hunter syndrome, often presents neurological manifestations. This study examines the differences between severe and mild Hunter syndrome and between central and peripheral nervous system as well as auditory and visual manifestations; it also looks at the alterations observed in magnetic resonance imaging of the head. DEVELOPMENT. The difficulties involved in distinguishing between severe and mild forms of Hunter syndrome, above all in the ‘intermediate’ phenotypic forms, are also discussed. The study also reports the most notable clinical manifestations and the diagnostic and therapeutic management of the cognitive and behavioural disorders, hydrocephalus, epileptic seizures, spinal cord compression, visual and hearing disorders, and carpal tunnel syndrome. Finally, the limited, heterogeneous findings from magnetic resonance imaging of the head are analysed.

CONCLUSIONS In severe Hunter syndrome, the lead factors during the period of neurodegeneration are cognitive disorders and conduct disorders. Hypoacusis and carpal tunnel syndrome are frequent in both severe and mild Hunter syndrome. Some complications (hypoacusis, hydrocephalus, spinal cord compression, carpal tunnel syndrome) offer a better prognosis if they are detected and treated at an early stage. Currently there is no clear correlation between neuroimaging alterations and the clinical features. Systematic study and monitoring, together with early symptomatic and enzyme treatment are likely to change the natural history of many patients with Hunter syndrome. KeywordsCarpal tunnel syndrome Cognitive compromise Conduct disorders Enzyme replacement therapy Epileptic seizures Hunter syndrome Hydrocephalus Hypoacusis Mucopolysaccharidosis II Myelopathy Ocular disorder CategoriesNeurología del Lenguaje y la Comunicación

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Neurological manifestations of Hunter syndrome