Summary
Neuroradiological findings in a 44-year-old male with the typical mild type of Hunter's disease are reported. Cranial MRI revealed patchy areas of increased and decreased signals in T1- and T2-weighted images in the thalamus and the basal ganglia giving rise to a honey comb-like appearance as a whole. The deep white matter showed high signals in the T2-weighted image. To our knowledge, the honey comb-like appearance has never been reported in this disorder. Deposition of mucopolysaccharides and/or glycolipids and increase in fluid content seem to be responsible for these changes.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Lichtenstein JR, Bilbrey GL, McKusick VA (1972) Clinical and probable genetic heterogeneity within mucopolysaccharidosis. II. Report of a family with a mild form. Johns Hopk Med J 131: 425–435
Wende S, Ludwig B, Kishikawa T, Rochel M, Gehler J (1984) The value of CT in diagnosis and prognosis of different inborn neurodegenerative disorders in childhood. J Neurol 231: 57–70
Watts RWE, Spellacy E, Kendall BE, Du Boulay G, Gibbs A (1981) Computed tomography studies on patients with mucopolysaccharidosis. Neuroradiology 21: 9–23
Ludwig B, Kishikawa T, Wende S, Rochel M Gehler J (1987) Cranial computed tomography in disorders of complex carbohydrate metabolism and related storage diseases. AJNR 4: 431–433
Kurkani MV, Williams JC, Yeakley JW, Andrews JL, McArdle CB, Narayama PA, Howell RR, Jones AJ (1987) Magnetic resonance imaging in the diagnosis of the cranio-cervical manifestations of the mucopolysaccharidosis. Magn Reson Imag 5: 317–323
Nakajima S, Tanaka A, Kawasaki H, Hattori H, Matsuoka O, Murata R, Isshiki G, Inoue Y (1988) Magnetic resonance imaging in mucopolysaccharidoses. No Hattatsu (Japanese) 20: 468–473
Dekabene AS, Constantopoulos G (1977) Mucopolysaccharidosis type I, II, IVA and V pathological and biochemical abnormalities in the neural and mesenchymal elements of the brain. Acta Neuropathol 39: 1–7
Nagashika K, Endo H, Sakakibara K, Konishi Y, Miyauchi K, Wdy JJ, Suzuki Y, Onisawa J (1976) Morphological and biochemical studies of a case of mucopolysaccharidosis II (Hunter's syndrome). Acta Pathol Jap 26: 115–132
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Shimoda-Matsubayashi, S., Kuru, Y., Sumie, H. et al. MRI findings in the mild type of mucopolysaccharidosis II (Hunter's syndrome). Neuroradiology 32, 328–330 (1990). https://doi.org/10.1007/BF00593056
Received:
Issue Date:
DOI: https://doi.org/10.1007/BF00593056