Summary
Neurological complications resulting from pronounced cerebrovascular changes were observed in a 59-year-old female suffering from Grönblad-Strandberg syndrome. In addition to typical skin changes of the pseudoxanthoma elasticum and ocular alterations (exudative macular degeneration and angioid streaks) there were an unusual number of disturbed endocrinological functions. The ophthalmological findings revealed characteristic breaks in Bruch's membrane, proliferation of chorioidal vessels into the subretinal space, hyperplasia of retinal pigment epithelium and disciform macular degeneration. Postmortem examination of the brain showed multiple cerebral infarcts due to recurrent disturbances in cerebral blood supply and particularly pronounced changes in the elastica interna of many cerebral arteries. Although the observed neurological signs in our patient were not characteristic of Grönblad-Strandberg syndrome, the histopathological findings led to the assumption that the same basic mechanisms result in alternations of elastic fibres and collagen, mainly in the ocular-cerebral system.
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References
Carlborg U, Ejrup B, Grönblad E, Lund F (1959) Vascular studies in pseudoxanthoma elasticum. Acta Med Scand 166 [Suppl 350]:1–84
Connor PJ, Juergens JL, Perry HO, Hollenhorst RW, Edwards JE (1961) Pseudoxanthoma elasticum and angioid streaks. Am J Med 30:537–543
Darier MJ (1869) Pseudoxanthome élastique. Ann Dermatol Syphiligr 7:1211–1212
Eddy DD, Farber EM (1962) Pseudoxanthoma elasticum. Internal manifestations: a report of cases and a statistical review of the literature. Arch Dermatol 86:729–740
Franceschetti A, Roulet EL (1935) Le syndrome de Groenblad et Strandberg (Stries angioides de la rétine et pseudoxanthome élastique) et ses rapports avec d'autres affections du mésenchyme. Bull Mem Soc Fr Ophtalmol 48:416–422
Galle G, Galle K, Huk W, Taghavy A (1981) Stenosierende Veränderungen der cerebralen Arterien bei Pseudoxanthoma elasticum. Arch Psychiatr Nervenkr 231:61–70
Gautier, JC (1981) Oral contraceptives and arterial strokes. Clinical data and a possible mechanism. In Greenhalgh RM (ed) Hormones and vascular diseases. Pitman Medical, London, pp 115–118
Goeminne L, van Ginneken E, Bernhard R (1963) Syndrome de Groenblad-Strandberg-Touraine (Elastorrhexie systématisée ou Elasto-dystrophie héréditaire). A propos d'un cas associant un état para-diabétique à une atteinte artérielle périphérique précoce. Presse Med 71:2511–2514
Goto K (1975) Involvement of central nervous system in pseudoxanthoma elasticum. Folia Psychiatr Neurol Jpn 29:263–277
Grönblad E (1932) Pseudoxanthoma elasticum and changes in the eye. Acta Derm Venereol (Stockh) 13:417–422
Iqbal A, Alter M, Lee SH (1978) Pseudoxanthoma elasticum: a review of neurological complications. Ann Neurol 4:18–20
Messis CP, Budzilovich GN (1970) Pseudoxanthoma elasticum. Report of an autopsied case with cerebral involvement. Neurology (Minneap) 20:703–709
Prick JJG (1938) Pontine pseudobulbar paralysis in Pseudoxanthoma elasticum. A clinical and anatomical study. (in Dutch). Gebr van Aelst, Maastricht
Strandberg J (1929) Pseudoxanthoma elasticum. Z Hautkr 31:689
Urbach E, Wolfram S (1938) Über Veränderungen des elastischen Gewebes bei einem autoptisch untersuchten Falle von Groenblad-Strandbergschem Syndrom. Arch Dermatol Syphiligr 176:167–175
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Fasshauer, K., Reimers, C.D., Gnau, H.J. et al. Neurological complications of Grönblad-Strandberg syndrome. J Neurol 231, 250–252 (1984). https://doi.org/10.1007/BF00313660
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DOI: https://doi.org/10.1007/BF00313660