The existence of thromboangiitis obliterans as a clinical entity has been a matter of debate for many years. In contrast to other immunovasculitides there is no organ involvement while peripheral vessels are affected. Heavy smokers under 40 years of age have a high predisposition for the disease. The cerebral form shows relapsing brain infarctions which can be visualized in CCT while panarteriography remains negative. Apart from unspecific inflammatory signs in blood and CSF there are distinctive laboratory findings proving the autoimmunological character of von Winiwarter-Buerger’s disease. In the serum anti-elastin antibodies, IgE and anticollagen antibody activity are detectable. In 3 patients the authors detected specific immunohistochemical findings in a biopsy specimen of the temporal artery. In addition to platelet-inhibiting substances corticoids in acute and azathioprine in chronic treatment becomes necessary.
We have investigated 6,972 patients with directional continuous-wave Doppler sonography within the last three and a half years, and have derived criteria for the sonographic diagnosis of basilar artery occlusion or tight stenosis in conjunction with 1,071 retrograde brachial angiograms. By sonographic patterns, we have suspected obstruction of the basilar artery or of both distal vertebral arteries in nine cases. Either bilateral sonographic silence or the absence of a diastolic flow component of the vertebral arteries served as criteria in the sonographic evaluation. Angiography of the vertebro-basilar system, performed in eight cases, showed near or complete occlusion in the distal vertebrals or in the proximal basilar artery. Degrees of stenosis less than an 80 percent reduction in lumen diameter could not be detected sonographically. Two further basilar artery occlusions were detected by means of angiography despite negative Doppler sonography: one of these patients showed an extensive collateral circulation between the posterior inferior and the superior cerebellar arteries, and one patient had an occlusion only of the middle and rostral thirds of the basilar artery, the proximal third and the anterior inferior cerebellar arteries being widely patent. Thus, we believe that directional CW Doppler sonography is very useful in the diagnosis of near or complete occlusion of both distal vertebral arteries or of the proximal basilar artery.
In six patients with ischemic lesions of the visual pathways above the lateral geniculate body, the pupil light reflex was investigated under perimetric conditions. The size of the lesions--in four cases caused by lateralized cerebral infarction, in two cases by complicated migraine attacks--was certified by neurological examination and by angiography or computerized tomography respectively. The recordings were done with a portable pupillograph measuring the infrared light reflected from the iris. A Tübinger perimeter served as stimulator. All patients except one, whose visual fields recovered quickly and completely, showed parallel tracings concerning the sensory and the pupillographic profiles, in the older lesions as well as in the recent ones.
The case history is presented of a man who died at the age of 38 years and had been suffering from severe torsion dystonia and a hypokinetic-rigid motor disturbance since the age of 10. The pathological findings were isolated pallidal degeneration with demyelination and moderate neuronal loss, iron pigment accumulation and spheroid bodies in both pallidal nuclei. In addition the zona reticularis of the substantia nigra was hypoplastic and not affected. The red nucleus, the dentate nucleus and the zona reticularis of the substantia nigra showed iron depigmentation. The rare condition of non-familial Hallervorden-Spatz syndrome, without involvement of the zona reticularis and the cerebral cortex, was considered to be the diagnosis.
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