Aspergillosis of ethmoid sinus and optic nerve, with arteritis and rupture of the internal carotid artery.

A 54-year-old woman had been treated 26 years previously for Hodgkin's disease. Eight months before presentation, the disease had recurred and the patient had received cytotoxic drugs and steroids. She presented with loss of vision of the right eye and died 5 months later from extensive subarachnoid hemorrhage. Postmortem examination revealed an aspergillus abscess of the ethmoid sinus, extending to the right frontal lobe and optic nerve, and to the wall of the right internal carotid artery (ICA). Death was due to rupture of the ICA. Such a presentation of aspergillosis is unusual. Three cases of aspergillus arteritis causing rupture of the ICA have been reported previously.

[Neuropathies of unknown etiology. A study of 48 cases]. / Les neuropathies de cause indéterminée. Etude de 48 cas.

A prospective study, carried out over 7 years, showed that out of 432 peripheral neuropathies followed up for 12 months, no cause could be discovered in 48 (11%). This is discussed with respect to the previous few reports. In fact none of these studies is strictly comparable. In spite of complementary investigations and observation of the course of the disease, it may be difficult to be sure to which etiology any individual case belongs; e.g. genetically determined neuropathies, chronic inflammatory polyradiculoneuritis or drug-induced neuropathies. However, it is clear that the group of neuropathies of undetermined origin, is clinically, pathologically and developmentally heterogeneous. This suggests that these patients should be classified as having a yet undetermined disease process. In our series, long term follow up enabled us finally to find a cause in a significant number of cases.

Case of dementia and myoclonia in an adult associated with anomalies in polyunsaturated fatty acids in leukocytes and peripheral nerve. An ultrastructural study of peripheral nerve.

We report a case of a 66-year-old patient presenting with abnormal movements and associated dementia. Death occurred 4 years after the onset of symptoms. In spite of the lack of autopsy results, the picture was one of late onset neuronal ceroid lipofuscinosis (Kufs' disease). Ultrastructural study of a peripheral nerve biopsy sample indicated a process of demyelination associated with unusual inclusions in Schwann cell cytoplasm. Biochemical analysis of the same sample and leukocytes showed considerable alterations in polyunsaturated fatty acid levels. These findings are discussed in the light of work on cases of infantile neuronal ceroid lipofuscinosis.

[Morphological effects of microinjections of Lewis rat serum into nerves of Lewis rats]. / Effets morphologiques de microinjections de sérum de rat Lewis dans le nerf de rat Lewis.

Recent reports have shown that intraneural injections of sera from patients with Guillain-Barré syndrome have demyelinating effects on rat peripheral nerve. Some authors have proposed that this could merely result from immune phenomena due to species differences. In this study we injected normal human sera and sera from normal Lewis rats into nerves of Lewis rats. We consider that the small lesions observed were due more to the injection procedure itself than to effects of the sera.

Familial Creutzfeldt-Jakob disease with extensive degeneration of white matter. Ultrastructure of peripheral nerve.

A case of Creutzfeldt-Jakob disease (CJD) in a 52-year-old man is described. At post mortem, extensive involvement of white matter was seen. A few similar cases have been reported mainly by Japanese authors. Our patients belonged to a French family in which 14 cases of CJD over three generations have been recorded. One of the patient's first cousins also had extensive white matter involvement. This is an unusual panencephalopathic form of CJD. The causes of the involvement of white matter are unknown. There was no clinical evidence of neuropathy but an electron-microscopic study of biopsied superficial peroneal nerve showed it to be present. Study of peripheral nerves is suggested for all patients with CJD.

Nonviral intranuclear inclusions in a nerve biopsy: an ultrastructural study.

During the ultrastructural study of a nerve biopsy from a diabetic patient suffering from chronic polyradiculoneuritis, we observed that a significant number of endoneural fibroblasts had abnormal nuclei. They contained a round, centrally located inclusion consisting of numerous irregularly arranged filaments approximately 15 nm in diameter. No such lesion in peripheral nerve has previously been reported. Interpretation of these abnormalities is difficult.

Coexistence of minicores, cores, and rods in the same muscle biopsy. A new example of mixed congenital myopathy.

The authors report on the ultrastructural study of a muscle biopsy carried out in a child 6 years after the first biopsy which had led to diagnosis of "multicore disease". Clinical evolution following a few years of muscular involvement had been favorable. The lesions observed in the second biopsy were again characterized by the presence of multicores, but in addition to cores and rods. All of these abnormalities could sometimes be noted in a single muscle fiber. Although rare cases of an association of two types of lesions have been reported, no study has hitherto shown multicores, cores, and rods in the same biopsy. This observation might confirm the possibility of common pathogenic mechanisms producing these lesions.