Inflammatory demyelinating lesions in two patients with IgM monoclonal gammopathy and polyneuropathy.

We report two patients with polyneuropathy and IgM monoclonal gammopathy in whom peripheral nerve biopsy showed the widening of myelin lamellae which is characteristic of IgM paraproteinaemic neuropathy. Moreover, certain myelinated fibres were invaded by histiocytes overloaded with myelin debris, and in some instances elongated macrophage processes could be seen peeling away the myelin lamellae. The latter ultrastructural features are characteristic of inflammatory demyelinating polyneuropathies in both human and experimental pathology. Such an association has not been reported to date in human pathology, but could explain the prevalence of inflammatory demyelinating lesions in experimental models of IgM paraproteinaemic neuropathy. These two cases seem to bridge the gap between inflammatory demyelinating polyneuropathies and polyneuropathies associated with IgM monoclonal gammopathy.

[Neurogenic muscular hypertrophy. Association with abnormal electrophysiological activities]. / Hypertrophie musculaire neurogène. Association à des activités électrophysiologiques anormales.

Muscle hypertrophy in neurogenic disorders is an uncommon phenomenon which has been reported in various conditions: hereditary or acquired anterior horn cell diseases, essentially S1 radicular compressions, and polyneuritis generally of a demyelinating type. We report two cases of denervating disease with muscle hypertrophy. The first was an S1 radiculopathy, and the second a compression with ischemia of the spinal cord by herniation of the D11-D12 disc, and showing partial improvement after surgery. In both cases, electrophysiological examination of the hypertrophic muscles revealed abnormal activities identified as complex repetitive discharges. In the second case they were associated with a syndrome of continuous motor unit activity. Such activities are rare in peripheral nerve involvement. However they have quite often been recorded in cases of neurogenic muscle hypertrophy and may therefore be partly responsible for the development of the hypertrophy.

[Post-radiotherapy anterior horn cell syndrome]. / Syndrome de la corne antérieure post-radiothérapique.

Three patients developed a progressive flaccid paraparesis without sensory or sphincter disturbances, following radiotherapy for lymphoma in two cases and carcinoma of testis in one case. The course was progressive with stabilization between two and four years. Electrophysiological study suggested anterior horn cell damage the mechanism of which remains unclear.

Reversible cerebral arteriopathy associated with the administration of ergot derivatives.

Three cases of intracranial arteriopathy associated with the administration of ergot derivatives are reported. In the first case, excessive doses of dihydroergotamine and of ergotamine tartrate seemed to be the cause, but simultaneous treatment with an antibiotic and the existence of a cytomegalovirus infection could have favoured the arteriopathic process. The second patient also received excessive doses of these two drugs, but in addition she was taking oestrogen and progesteron containing contraceptives. In the third case, an intravenous injection of methylergometrine in a dose of 0.2 mg seemed to have initiated the arteriopathy. Arteriograms were comparable in all three cases, with segmental stenosis of several cerebral arteries, whereas internal carotid and vertebral arteries remained normal. In the first case a control cerebral arteriography was normal one month later. These findings suggest that widespread cerebral arteriopathy can appear in subjects who chronically abuse ergotamine tartrate.

[Allergic granulomatous angiitis (Churg-Strauss syndrome) manifested as polyneuritis. Its relation to panarteritis nodosa]. / Angéite granulomateuse allergique (syndrome de Churg et Strauss) révélée par une multinévrite. Ses rapports avec la panartérite noueuse.

A 54 year old man with a history of resistant asthma presented with a multiple neuritis of subacute onset and hypereosinophilia. Histology showed necrotizing vasculitis with multinucleated giant cells compatible with Churg and Strauss' syndrome. Ultrastructural examination of nerves and immunopathological tests showed anti IgG serum fixing deposits in the arterial wall. Recovery was complete after corticoid therapy for 20 months. Literature on the Churg and Strauss' syndrome is reviewed and possible clinical, biologic, histopathologic, and physiopathologic relations between allergic granulomatous angiitis and panarteritis nodosa are discussed.

[Neurosyphilitic arteritis. Clinical, paraclinical and therapeutic data. A review of six cases (author's transl)]. / Neuro-vascularites syphilitiques. Donnees cliniques, paracliniques et thérapeutiques. A propos de six observations.

Among forty-six patients with neurosyphilis, seen over a ten-year period, six had features highly suggestive of syphilitic cerebral vascularitis. The carotid distribution was involved in five patients and the posterior cerebral artery in one. As exemplified by these six observations, syphilitic arteritis should be considered when an ischemic stroke occurs with no apparent etiology. The fluorescent treponemal antibody adsorbed test (FTA-ABS) in the cerebrospinal fluid is the most reliable and the most specific laboratory test for the diagnosis of neurosyphilis.

[Cerebral emboli due to subclinical heart disease. Value of thorough investigations (author's transl)]. / Embolies cérébrales dues à des cardiopathies cliniquement inapparentes. Intérêt d'une attitude investigatrice active.

In a series of 250 cases of cerebral vascular accident, the authors have selected 12 patients whose embolus appeared to have originated in the heart, although this could not be confirmed by clinical examination, ECG, Holter system monitoring and echocardiographic studies. Angiocardiography, complemented or not by His bundle exploration and/or coronary arteriography, revealed the presence of a heart disease likely to produce emboli in 11 cases, and in 8 cases, this was prolapsed mitral valve. These 11 cases represent 4.5% of the whole series and 22% of cases with emboli of suspected cardiac origin. Thorough cardiological studies, therefore, seem to be justified in young adults presenting with stroke. The high incidence of prolapsed mitral valve is in keeping with recently published data.

[Multiple affections of the cranial nerves as a pathognomonic sign of periarteritis nodosa. Relationship with Cogna's syndrome (author's transl)]. / Atteinte multiple des nerfs crâniens révélant une périartérite noueuse. Relation avec le syndrome de Cogan.

A 43-year-old woman was found to have multiple cranial neuritis affections, present as an isolated disorder over a long period, and characterized by bilateral deafness and associated lesions of the VII and V cranial nerves. On investigation, visual disturbances were discovered which were of a transitory nature and made up a total clinical picture suggesting Cogan's syndrome. Neuropathological examination revealed the presence of typical periarteritis nodosa lesions in the cranial nerves. The authors suggest, therefore, that certain cases described as Cogan's syndrome are in fact particular forms of periarteritis nodosa.