[Supratentorial hemangioblastoma].

The authors report the case of an isolated supratentorial hemangioblastoma occurring in a woman operated on for a cerebellar hemangioblastoma twenty years before. The data furnished by immunohistochemical technics don't seem to be deciding and don't work out the problem of hemangioblastoma histogenesis.

[Cerebral neuroblastoma in the newborn infant].

The authors report the exceptional case of a cerebral neuroblastoma responsible for intracranial hypertension at birth. In spite of neurosurgical intervention on the seventh day the newborn died. Histological and immunohistochemical study with Protein S 100 and "anti LEU 7" are consistent with the diagnosis of neuroblastoma.

[Generalized mucormycosis. Apropos of a case caused by Absidia corymbifera].

This is the case of a woman, 49 year old, suffering from acute lymphoblastic common leukemia, who died from respiratory and cardiac insufficiency 47 days after the onset of aplasia. Autopsy revealed cardiac and cerebral dissemination of pulmonary mycosis. Absidia corymbifera (Mucoraceae, Phycomycete) was shown by culture to be the pathogen involved.

Plasmacytoma and gastric amyloidosis associated with nodular pulmonary amyloidosis.

The authors report an association of gastric and pulmonary amyloidosis in a 74 year old woman. The patient had had a gastric plasmacytoma for the last 11 years. On autopsy, the stomach was found to contain intramural deposits of amyloid, and was infiltrated by plasma cells secreting monoclonal IgG lambda light chain.

[An adult case of Leigh's subacute necrotizing encephalomyelopathy].

Leigh's encephalomyelopathy has been mainly observed in infancy and childhood. A later onset, during adolescence or adulthood has been rarely reported. Our patient was a 35 year-old man who died after 10 months of evolution of a subacute neurological syndrome, beginning with behavioural changes then a confusional state, epileptic fits, ataxia, autonomic disorders, abnormal alimentary behaviour and dementia.

[Peripheral neuropathies disclosing sarcoidosis].

A 54 year-old woman presented with an asymmetrical polyneuropathy, with in addition signs of hepatitis and hypercalcemia. The diagnosis of sarcoidosis was made by hepatic and neuromuscular biopsies. Electrophysiological studies showed an asymmetrical clear neurogenic involvement with an axonal pattern.

Peripheral neuropathy with essential mixed cryoglobulinemia: biopsies from 5 cases.

Essential mixed cryoglobulinemia, which can cause hypersensitivity vasculitis, was observed in five patients with peripheral neuropathy. Three cases presented with multifocal neuropathies and two cases with symmetrical polyneuropathy. One had cryoglobulinemia with IgM monoclonal gammopathy IgG polyclonal gammopathy, and the other four had cryoglobulinemia with polyclonal gammopathy.

The C57BL mice: an animal model for inflammatory demyelinating polyneuropathy.

Recently it has been reported that the C57BL mice can be used as a model of benign monoclonal gammopathy (MG). Since experimental models have until now failed to reproduce specific lesions of human dysglobulinemic polyneuropathies, we decided to investigate the peripheral nerve of these mice. The sciatic nerve and the serum from 14 C57BL mice were examined: 9 of these animals were found to have an IgG, kappa light chain MG, while in the other 5 no abnormalities were detected in the serum.

[Neurogenic muscular hypertrophy. Association with abnormal electrophysiological activities].

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.

Guillain-Barré syndrome: an ultrastructural study of peripheral nerve in 65 patients.

65 biopsies of peripheral nerve from patients suffering from Guillain-Barré syndrome were studied by electron microscopy. In 48 cases there was macrophagic invasion of the Schwann cells of certain myelinated fibers, and in 32 of these cases some myelin sheaths were stripped away by an elongated macrophagic process. Vesicular disruption of the myelin sheath was observed in only 8 cases and in less than 1% of the myelinated fibers.

[Divry-Van Bogaert cortico-meningeal angiomatosis and Sneddon's syndrome. Nosological study. Apropos of 4 cases].

Four patients with idiopathic livedo reticularis and multiple cerebral ischemic events leading to a severe dementia are reported. Imaging (CT, MRI, angiograms) and pathological features (in one case) are presented. Similar clinical features have been described previously in Divry-van Bogaert's disease and Sneddon's syndrome.

[Polyneuropathies during prolonged stays in resuscitation].

Extensive multiple neuropathies were observed in four patients after prolonged stay in intensive care units. Date of onset of the multiple disorders was difficult to determine due to disturbed consciousness of all patients during the first few weeks of intensive care: it was probably between the 10th and 30th days. Electrophysiological examinations in the 4 patients, and ultrastructural study of musculocutaneous nerve of leg in 2 cases, suggested an acute axonal lesion.

Uncompacted lamellae in three patients with POEMS syndrome.

Three patients with POEMS syndrome were studied: two of them had myeloma, whereas the third had M protein without myeloma. Peripheral nerve biopsies showed no deposits of anti-Ig sera at direct immunopathological examination. Ultrastructural study revealed myelino-axonal degeneration in all three cases.

Carcinomatous neuropathy. An ultrastructural study of ten cases.

Superficial peroneal nerve biopsies were studied from 10 patients with carcinomatous neuropathy. There were 6 patients with the sensorimotor form and 4 patients with the sensory form. In both forms of neuropathy, histometric studies showed a variable loss of myelinated fibers which was generally most severe in the sensory form.

Variability of morphological features in early infantile polyneuropathy with defective myelination.

Four cases of early infantile polyneuropathy with defective myelination are reported. The peripheral nerve was studied by light and electron microscopy; different morphological characteristics have been noticed in these patients. Case 1 presented aspects of defective myelination with atypical "onion bulb" formation composed of multiple layers of basement membrane.

Quantitative, histological and ultrastructural studies of peripheral nerve in arteriosclerotic non-diabetic patients.

The superficial peroneal nerve was taken from 12 arteriosclerotic non-diabetic patients just after amputation of a leg. Preparations of teased fibers were performed in 8 cases. Specimens were studied by light and electron microscopy in all cases.

Relapsing inflammatory demyelinating polyneuropathy in a diabetic patient.

Inflammatory demyelinating polyradiculoneuropathies exhibit well-known ultrastructural lesions of the peripheral nerve, both in acute cases, i.e., Guillain-Barré syndrome, and in relapsing, sub-acute and chronic cases.

[Tomaculous neuropathy. Electrophysiologic study].

In two cases with recurrent palsies, the results of electrophysiological studies led to nerve biopsy showing typical tomacula. The first case was an inherited neuropathy with liability to pressure palsies. The second case was an apparently sporadic painless recurrent brachial neuropathy.

Waldenström's macroglobulinemia and peripheral neuropathy: deposition of M-component and kappa light chain in the endoneurium.

Some cases of peripheral neuropathy associated with benign IgM monoclonal gammopathy, or Waldenström's macroglobulinemia, are probably of autoimmune origin; in some cases, anti-IgM serum reacts with the myelin sheaths of peripheral nerves. However, mechanisms may differ in other cases. In one case of neuropathy with macroglobulinemia, we found deposits of IgM immunoglobulin in the endoneurium.