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We report a case of an adenomatoid tumor of particular location within the pleura, incidentally discovered on a pulmonary lobectomy specimen after surgical resection of a pulmonary squamous cell carcinoma. This adenomatoid tumor appeared as a unique pleural mass located away from the primary carcinoma and consisted of a cellular proliferation organised in tubes and sheets. Adenomatoid tumors are considered as benign tumors of mesothelial nature. Their morphological and immunohistochemical features in association with their location to the pleura, warrant a precise analysis to eliminate malignant tumours such as malignant mesothelioma or metastatic adenocarcinoma.

Papillary tumors of the temporal bone are rare and aggressive neoplasms. Recently described, these tumors had initially a presumed middle-ear origin. Only recently, convincing anatomic, morphological and immunohistochemical arguments exist for an endolymphatic sac origin (inner-ear origin). We report two cases of endolymphatic sac tumor. These tumors can be encountered sporadically or in Von Hippel-Lindau disease. They classically grow very slowly, resulting in late clinical manifestations with expansive mass invading temporal bone and extending in posterior fossa. Radiologically, these endolymphatic sac tumors can mimic metastatic carcinoma, paraganglioma, or cerebellar haemangioblastoma specially in von Hippel-Lindau disease. Histology shows a papillary epithelial tumor with hypervascular stroma, without atypia. The treatment for these tumors is surgical and curative when early diagnosed. In apparently sporadic cases, genetic analysis for Von Hippel-Lindau disease should be considered.

Cytomegalovirus (CMV) infection of the nervous system is frequent in acquired immunodeficiency syndrome (AIDS) and can be responsible for encephalitis, encephalomyelitis, meningoradiculitis or polyradiculo-neuropathy. Encephalitis is characterized at microscopy by its periventricular and cerebellar location, and by the presence of cytomegalic cells, containing intranuclear and/or intracytoplasmic inclusions, microglial nodules and necrotic foci. The virus can infect almost all types of cells. Coexistence of CMV and HIV has been observed in giant cells of macrophagic origin. It has been suggested that the two viruses could act in synergy. The nervous system is seldom infected by the varicella-zoster virus (VZV) in AIDS. The infection can be responsible for multifocal leukoencephalitis, ventriculitis, vascular lesions associated or not with cerebral infarction, or with meningomyeloradiculitis. In almost all cell types Cowdry's type A intranuclear inclusions have been found. The virus can be demonstrated by immunohistochemistry or in situ hybridization. VZV antigens have been reported in the walls of vessels damaged by a non inflammatory obliterating vasculopathy or by a granulomatous angiitis. Coexistence of VZV and HIV has been observed in giant cells of macrophagic origin, and synergy between those two viruses has been suspected.

Sinusonasal polyposis is frequent but usually benign. A 77-year-old woman with an history of sinusonasal polyps, presented with fever and neurologic impairment. Radiologic imaging showed a polypoïd mass filling the nose and sinuses, eroding the ethmoïd bone, progressing intracranially, and causing brain abscess. The literature is reviewed, according to the relations between brain abscess development and nasal polyposis, with emphasis on intracranial extension of sinusonasal polyps. Treatment of the nasal polyps, usually based on the use of local or general corticosteroïds, can avoid this serious complication.

We report the natural history of 17 brain lymphomas (11 primary, 6 disseminated) from a post-mortem series of 130 patients with AIDS. Primary lymphomas appeared lately in the course of AIDS. They were often associated with a severe T-cell immunodepression and with more frequent opportunistic disorders than disseminated lymphomas. Associated Kaposi's sarcomas were surprisingly frequent. All patients presented with neurological manifestations. Heterogeneous features were seen at CT examination. The CSF was abnormal in 12/13 cases, with an increase of protein contents and secretion of immunoglobulins; it contained activated lymphocytes in 5/6 cases of disseminated lymphomas, and malignant cells in only one case. Cellular density never exceeded 8/mm3 for primary lymphomas, and the lymphocytes were considered normal. The pre-mortem diagnosis of cerebral lymphomas was made in five patients, with a time lapse of 1 to 7 months between the first neurological symptoms and death, and of 5 to 30 days between the diagnosis and death. Cerebral biopsy was diagnostic in 4 cases of primary cerebral lymphomas. In only 1/6 patients with disseminated lymphomas, the diagnosis had been made when the patient was still alive, based on CSF and bone marrow lymphomatous infiltrations. The diagnosis of cerebral lymphoma (7 primary, 5 disseminated) was post-mortem in 12 cases. It was made only at microscopic examination in 2/12 cases of primary lymphomas. The histopathological study frequently showed a multicentric involvement, and always an immunoblastic cell type with plasmablastic differentiation and frequent medium size cells. Marked gliosis and significant necrosis were often observed. Neuropathological lesions associated with HIV-1 infection (toxoplasmosis, CMV and HIV-1 encephalitis) were seen in 8 cases with primary lymphomas.

A case of malignant melanoma discovered at the base of the skull is reported in a 52-year-old male. The patient with no previous significant history, complained of headaches. He developed progressive paralysis of the IX, X and XI left cranial nerves and a Claude-Bernard-Horner syndrome. The tumor, discovered at the nervous compartment of the jugular foramen was treated by surgery and radiotherapy. The patient died 27 months after surgery. The absence of other systemic localisations allows to consider this melanoma as primitive. The presence of spindle cell areas in the tumor may suggest the diagnosis of melanotic schwannoma. Immunohistochemistry is still disappointing because of the lack of specific markers. Our results, in agreement with those of the literature, emphasize the importance of the histopathological findings and the determining role of the electron microscopy in the diagnosis and the differential diagnosis of these two entities, whose nosological frontiers may, sometimes, be difficult to distinguish.

Five cases of Fischer's "one and a half" syndrome are reported. A complex central ophthalmoplegia provokes paralysis of horizontal eye movements on the affected side in both eyes and an internuclear ophthalmoplegia on the same side. No horizontal movement, except in convergence, is possible in the eye on the affected side, the other eye exhibiting movement between the median line and abduction only. Three patients with this syndrome were examined by oculography. The outcome was fatal in one case, pathological examination demonstrating widespread damage to half the pontine protuberance. In another case scanner imaging showed a paramedian protuberential lesion. The syndrome is most frequently observed in multiple sclerosis and cerebrovascular accidents but may follow any lesion in this region. Physiopathological features of the disorder are discussed.

An immunological mechanism may be responsible for the peripheral neuropathies related to IgM monoclonal gammopathies. Myelin-associated glycoprotein (MAG) has been found to be one the main target antigens for these paraproteinemias. Glycosphingolipids of peripheral nerve have been also found to be targets for these antibodies, especially a sulfated glycosphingolipid containing glucuronic acid (GLSG) specific for peripheral nerve. Crossed reactivity has been found between MAG and GLSG. Anti-glycolipid antibodies were determined in 14 patients with IgM gammopathy and polyneuropathy. Glycosphingolipids from human peripheral nerve were purified and chromatographed by thin-layer chromatography for immunodetection of the antibodies. The 12 patients who had GLSG antibody activity had a clinical status identical to the reported cases of dysglobulinemic neuropathies with anti-MAG antibodies.

A patient developed a neurological disorder at the age of 57 years and died 9 years later after progressive aggravation. The clinical picture was that of a unilateral, parietal, focal neurological syndrome for many years, finally becoming bilateral. The purely neurological nature of the condition, without signs of mental disturbance, should be emphasized. Pathological examination showed mainly unilateral circumscribed cortical atrophy in the parietal regions. Histological examination showed atrophic lesions typical of Pick's disease. This case provides suggestive evidence for the existence of purely parietal forms of Pick's disease.

A right-handed 77-year-old man presented with a left homonymous lateral hemianopsia involving the macula. Consciousness, memory, and oral language were normal, but the patient experienced great difficulties in identifying objects and images by sight, even though he recognized their geometrical shapes. He also had prosopagnosia, and alexia without agraphia but no agnosia for colours. The patient died suddenly 6 weeks after the onset of the disorders. Pathological examination demonstrated the presence of a bilateral occipital infarct involving on the right, the inferior border of the calcarine fissure, and the lingual and fusiform gyri, and on the left the fusiform gyrus. This supports the role in man of the inferior occipitotemporal convolutions in the perceptual integration of visual informations, and suggests that this region of the cortex is analogous with the inferotemporal cortex of monkeys which has been shown to be a determinant for the elaboration of visual objects.

Clinical and pathological findings in three adults with toxoplasmosis of the central nervous system are reported. Symptoms and signs in the first patient, a Haitian woman who had lived in France for 2 years, were suggestive of a right hemispheric space-occupying process. The second case was a woman treated for Hodgkin's disease who showed symptoms and signs of a meningoencephalitis. The presenting lesion in case 3 mimicked a tumor of the posterior fossa. Analysis of these 3 cases and of those previously reported underlines: 1) diagnostic difficulties, particularly in immunodepressed patients; 2) the effectiveness of immunoperoxidase for pathological diagnosis; 3) the poor prognosis due to absence of a specific treatment.

Clinical and histopathologic study of central nervous system (CNS) was performed in 46 acute myeloid leukemia (AML) and 16 chronic granulocytic leukemia in the blastic phase (CGL). Involvement of the CNS developed in 28 cases. Eighteen patients out of these 28 had neurological symptoms. The frequency of meningeal leukemia depends on the number of lumbar punctures and on the survival time. Post-mortem examination was performed on 45 patients. Eighteen had evidence of CNS leukemic infiltration (18/45 arachnoid, 10/31 dura, 5/45 brain). Hemorrhages are frequent even without CNS involvement (19/27). CNS leukemic infiltration is common enough in AML and CGL to justify agressive diagnostic, therapeutic, and prophylactic measures.

The authors report a case of centropontine myelinosis associated with demyelinising lesions of the tectum mesencephali, the cerebellum, the central grey nuclei and the white matter of the hemispheres. This case is compared with similar observations already studied in the literature. From an aetiological standpoint, attention is drawn to the apparent causative role of diuretic therapy which had resulted in severe water and electrolyte disturbances.

The clinical picture in three cases of chronic cranial pachymeningitis of unknown origin was dominated by headache, disturbed balance, a confusional state and cranial nerve lesions. The erythrocyte sedimentation rate was increased and the CSF showed inflammatory changes. CT scan imaging showed thickening of the tentorium cerebelli, which took up contrast intensely. Meningeal biopsy showed the dura-mater to be the site of a non-specific inflammatory process. No precise cause was found. Clinical manifestations in these three patients were remarkably corticosensitive but lesions did not regress on CT. The development of a state of corticodependence led to an attempt at treatment with radiotherapy and/or azathioprine, but follow up is insufficient to evaluate results.

The authors report a case of hypertonia, the clinical and electrophysiological features of which were identical to those of the Stiff-man syndrome first described by Moersch and Woltman in 1956. This and the five other previously reported cases can be attributed to encephalomyelitis of the anterior horns of the spinal cord. All of these cases had severe but partial involvement of the anterior horns and the probable physiopathological mechanism of the resulting contractures was elective destruction of the small inhibitory neurones. In the present case, the association of a limbic encephalitis and secondary exteriorization of a carcinoma of the pharynx, strongly suggested a paraneoplasic encephalomyelitis. This is the first report indicating an association between the Stiff man syndrome and a paraneoplastic syndrome. Another point of interest was the spectacular response of the hypertonia to steroid therapy suggesting a central neuropharmacological effect quite apart from the non specific anti-inflammatory action on the encephalomyelitis.

Sixteen cases of anoxic encephalopathy in adult patients admitted to the intensive Care Department of Raymond Poincaré Hospital (Garches) have been selected on the following criteria: resuscitation by external cardiac massage for cardio-respiratory failure, without any previous history of anoxia and with detailed data on the subsequent evolution of neurological signs and symptoms. Main clinical features were compared to the results of the neuropathological examination. The pattern and the distribution of changes are detailed. The cerebral cortex was always involved by diffuse ischemic cell changes or by laminar necrosis. The Sommer's sector of Ammon's horn was affected in 15 cases, the basal ganglia in 14, the cerebellum in 11, the brain stem in 6. The very rarely mentionned necrotic brain stem changes, which involved inferior colliculi, periaqueductal gray matter and substantia nigra were sometimes associated with lesions of the floor of the IVth Ventricle and of the mamillary bodies. They were different from Wernicke's encephalopathy with could be dismissed on clinical and pathological grounds. No clinical signs or symptoms specific for this distribution of changes could be found by the retrospective study of the clinical files.

The authors report the anatomical and clinical findings in a 65 year old patient with a lesion in the hypothalamus presenting mainly as disorders in heat and water regulation. Disorders in heat regulation dominated the clinical picture, in relation to a poikilothermia. Conservation of reactivity to pyrogenic bacteria is debatable. Inappropriate secretion of antidiuretic hormone (ISADH) by a direct effect on the supraoptico-hypophyseal aixs can account for the water and electrolyte disturbances. The anatomical lesions, due to hypothalamic changes, can be included in the circumscribed proliferative reticuloses of the CNS having a pseudo-inflammatory histological appearance.

Assessments vary as to the contribution of the right hemisphere to language in aphasic patients. Results of a clinicopathologic study in two right-handed subjects with aphasia are reported. The lesions involved the territories of the left middle and anterior cerebral arteries. Oral productions in one case, studied during the three weeks of survival, were limited to automatic series, recitation of a fable, and completion of sentences. Follow-up in the other case was possible during 2 years of language rehabilitation. At the end of this period, repetition of an echolalic type was possible; a propositional expression had appeared, reduced to substantives and verb infinitives; denomination was possible but rich in semantic paraphasias. A second infarction in the right sylvian region caused the recovered language to disappear and to be replaced by an abolition of all communication. Proof was thus obtained that the progress accomplished was dependent on the right hemisphere. These findings are discussed in the light of observations of patients following left hemispherectomy and of the capacity of the right hemisphere to generate language as demonstrated in patients after commissurotomy. A dynamic interpretation of the taking over of expression by the right hemisphere during some aphasias is proposed.

Sarcomatous changes in Paget's disease are known to occur, but cranial localization with invasion of cerebral parenchyma is rarely seen. A case is reported of osteogenic sarcoma occurring during Paget's disease, which was diagnosed from neurological signs, and on which a complete anatomical study was made.

The use of a floating graft is proposed in order to attempt resolution of surgical problems raised by distal internal carotid artery lesions located high in the neck. Surgical treatment is essential, but vascular suture is impossible below the petrosa and ligature of internal carotid artery is often the only solution. The floating graft, which is fixed proximally only and is allowed to float in the circulation, allows exclusion of the lesion and maintenance of the carotid vascular axis. Preliminary experimental studies in the rat have provided satisfactory results and research should be continued in larger animals prior to possible clinical use.

A device using a Macintosh microcomputer and a light microscope equipped with a drawing tube is described. The mirror of the drawing tube is turned to the screen of the microcomputer. An example of its use in morphometry of peripheral nerves is given. At a 1000X magnification, the resolution of the system is 10 pixels per 2.6-microns. The coefficient of variation between 20 measurements of diameter of thin (3 microns in diameter) and large (12 microns in diameter) myelinated fibers was 8% and 2% respectively. This precise and inexpensive system could be easily run in laboratories of morphology.

The author choroidal artery syndrome is a rare disorder associating, in its complete form, a hemiplegia, a hemianesthesia, and a homonymous lateral hemianopia. Four cases are reported in which the diagnosis was confirmed by a CT scan, which demonstrated a low density area in the posterior part of the posterior limb of the internal capsule, sparing the thalamus medially, and extending laterally to the apex of the pallidum thus corresponding to the area supplied by the anterior choroidal artery. The incomplete forms of this syndrome and clinicopathological correlations are discussed.