Biomedical subjects
Publications and source records attributed to J Mikol.
The association of Crohn's disease and peripheral neuropathy is a rare event and the pathogenic factors often implicated are vitamin B12 deficiency or metronidazole treatment. We report a case of severe axonal polyneuropathy associated with Crohn's disease and unrelated to vitamin deficiency or metronidazole treatment. This represents a very rare extra-digestive manifestation of Crohn's disease.
We report the eleventh illustrated case of primary liposarcoma of the heart in a 28-year-old man. The tumor arose from the mitral valve and the left ventricle and was revealed by a solitary brain metastasis 1 year before. The primary tumor and the metastasis were surgically removed. The cardiac and brain neoplasms were myxoid liposarcoma, as confirmed by histochemical, immunohistochemical, and ultrastructural studies. Although review of the literature points out the poor survival of patients with malignant liposarcoma of the heart, no recurrence or metastasis was observed after a 6-month follow-up.
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A case of osteochondroma arising from the C2 transverse process, responsible for vertebral artery compression with complete occlusion and C2 nerve root irritation with C2 neuralgia, is reported. Complete surgical removal was achieved via the lateral anterior approach.
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Three siblings, a male and two females, respectively 47, 51 and 60 years-old, were investigated, since they had, like their father, labioglossopharyngeal paralysis, fasciculations and predominant cervico-facial muscular atrophy. No pyramidal signs were present. The disorder was fatal within 9, 5 and 14 months. In two cases the pathological examination showed a selective involvement of the peripheral motor neuron whereas the central motor neuron was normal. The main abnormalities consisted of abundant chromatolytic neurons and cell loss and were mostly present in the brain stem motor neurons. Immunocytochemical studies with antibodies against neurofilaments and protein-tau, and electron microscopic technics were performed on cervical motor neurons. Neurogenic muscular atrophy was massive in the tongue, less predominant in the diaphragm and psoas and were similar to lesions present in the peroneus brevis muscle. Transmission was autosomal dominant. We suggest that this clinico-pathological syndrome should be considered as a variety of the adult bulbo-spinal muscular atrophies.
Cerebral infarcts in 3 patients revealed the presence of disseminated intravascular coagulation (DIVC) of cancerous origin before any clinical manifestations of the neoplasm. Neurologic manifestations of these consumption coagulopathies almost constantly produce a picture of diffuse encephalopathy, expression of disseminated microinfarcts; however, transient or constituted focalized ischemic accidents by occlusion of a medium sized artery are also possible, and this in the absence of non-bacterial thrombotic endocarditis. Biologic diagnosis of DIVC is not always simple, and screening tests (platelet count, prothrombin and fibrinogen levels) can remain within normal limits during chronic forms, as a result of a subjacent inflammatory syndrome, frequently associated with cancer. Two other specific serum tests are therefore of fundamental interest: assay of fibrin degradation products and tests for soluble complexes.
The immunoreactivity of polyclonal antiserum to neuron-specific enolase (NSE) has been investigated. Twenty-three cases of malignant lymphoma (ML) were studied and compared with previously published reports. In our study 11 out of 23 cases showed strong or weak NSE positivity; any type of ML could be positive or negative even among B or T cell ML. This study indicated that polyclonal NSE is not a specific marker; it might be an inconstant marker of ML with no apparent correlation between reactivity and morphology or phenotype.
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Human immunodeficiency virus (HIV) RNA and proteins were detected in the brains of several AIDS patients with subacute encephalitis, by in situ hybridization and immunohistology. The majority of infected cells were mononucleated and bore processes. Using single and double immunohistologic procedures, the authors identified these cells as macrophages. The majority of them had the phenotype of microglial cells (Leu-M3-, CD4-), others were labeled with markers of circulating macrophages (Leu-M3+, CD4+/-). The presence of HIV RNA and proteins in CD4- cells could be explained by depressed CD4 antigen expression, as a result of infection or macrophage tissue differentiation.
A new familial syndrome that affected 3 of 7 siblings is described. All 3 patients were young women with a very peculiar phenotype, poikilodermia and hair greying, and idiopathic nonarteriosclerotic cerebral calcifications. Pathological studies demonstrated a marked and progressive hyalinosis involving capillaries and often arterioles and small veins of the digestive tract, kidneys, and calcified areas of the brain. Using electron microscopy, we found that the hyalin substance in the intestinal capillaries consisted of several concentric layers of basal membrane-like deposits within a finely granular fluffy material. Huge deposits of this material were present in the subepithelial and mesangial spaces of the kidneys. Endothelial cells and, in the kidneys, mesangial cells were markedly abnormal, and a true mesangiolysis pattern was present in 2 patients. The clinical and biologic expression of these vascular changes was variable. Diarrhea, rectal bleeding, malabsorption, and protein-losing enteropathy were the main and lethal clinical problems in the proband. Hypertension appeared in the early stage of a second pregnancy in 1 sister, and mild proteinuria was found in all 3 affected patients. Peripheral retinal ischemic syndrome and chorioretinal scars were found in the ocular fundi of both affected sisters of the proband. A subarachnoid hemorrhage, due to a right sylvian aneurism, also occurred in both sisters and was lethal in 1 sister. None of the known causes of distal vessel hyalinosis could be ascertained.
An unusual case of familial multisystemic degeneration is reported. Two siblings had juvenile parkinsonism, areflexia, and retinal degeneration of slow progression. The main neuropathological findings in case 1 were pallidoluysian, nigral, dentate, and dorsal columns degeneration. The authors draw a comparison between this case and juvenile parkinsonism, dentato-rubro-pallido-luysian atrophy, and spino-cerebello-nigral degeneration.
15 extensive glomus jugulare tumors have been studied. Optic and ultrastructural data were very similar to those which have been reported in the literature. They possessed a local aggressiveness, demonstrated by progressive spreading in soft tissues and bone, intravascular digitations and destruction of cranial or sympathetic nerves. Dura-mater was usually preserved and prevented extension close to the brain stem. In this group, no functional activity of catecholamine-secreting tumor nor metastasis proving a malignant form were observed.
Controversies exist about the type of lesions observed in Korsakoff's syndrome or amnestic syndromes. A review of clinicopathologic data in the literature and findings in a personal series of 31 patients with amnestic syndromes (14 with alcoholism and nutritional deficiency, 8 with tumors including 2 with craniopharyngiomas, 5 with Pick's disease and presbyophrenic manifestations, 2 with vascular affections and one each with anoxia and herpetic encephalitis) were used to determine most frequently responsible lesions. Although it is generally accepted that there must be bilateral lesions divergent opinions are found with regard to the significance of limbic-hippocampomammillary circuit lesions. Some authors refute any specific role for the limbic circuit, and particularly any part played by Ammon's horn, in favor of a role for the temporal isthmus while others reject the possible role of the mammillary bodies and implicate the dorsomedian nucleus of the thalamus. The present study confirmed the importance of the limbic-hippocampomammillocingulus circuit in the maintenance of long-term memory. Involvement of the hippocampus appears obvious, particularly in disorders due to lesions of the subiculum and sommer's field. Mammillary body lesions are a constant finding in amnestic syndromes due to alcoholic nutritional deficiency and may also be present in Korsakoff's syndromes of tumoral origin e.g. a compression due to a craniopharyngioma. The rarely mentioned lesions of the cingular convolution are a further possible cause. The role of a thalamic lesion has not been totally confirmed, findings indicating equally frequent involvement of laterodorsal and dorsomedian nuclei. Clinical evidence, however, points to correlations between fabulation, false recognition and a thalamic lesion. The role of the fornix is debatable, although it is difficult to understand how destruction of the hippocampic formation or of the mammillary bodies can provoke memory disorders while an interruption in the structure that links them would have no consequences, unless it is admitted that as in animals other pathways exist that pass little or not at all through the fornix. Clinical expression of bilateral lesions of these structures differs according to whether they affect the posterior Ammon's horn region or lie more anteriorly in the trigonomammillothalamo-cingular complex. Clinical features of hippocampic amnesia include continuous anterograde amnesia with successive periods of forgetfulness, retrograde deficit of variable duration, parallel alterations of verbal and visuospatial memory and absence of fabulation.(ABSTRACT TRUNCATED AT 400 WORDS)
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Efferent pathways of the LD nucleus of the thalamus were studied in 6 Papio-papio baboons with the retrograde transport technique utilizing HRP. Injections were made in cingular and parietal cortex and hippocampal formation. Large projection from the LD to the cingular and subicular cortex were visualized as reciprocal connections. No pathway to parietal area 7 was found. The course of the fibers is via the fornix, the cingular bundle and the retro-lenticular portion of the internal capsule. The result of this study make it necessary to reconsider neuropathological hypotheses on memory.
An X-linked spinal muscular atrophy is reported in one family. Four of the five patients were examined. In three, electromyography, conduction nerve velocities and muscle biopsy were consistent with anterior horn cell disease. Similar families in the literature were reviewed and characteristic data were: 1) adult-onset, 2) proximal, bulbar and facial involvement, 3) prominent perioral fasciculations, 4) frequent association of cramps, tremor and sexual dysfunction (hypogonadism and gynecomastia), 5) very slow progression and favorable prognosis. These characteristics define, among the spinal muscular atrophies, a distinct entity named by several authors Kennedy's disease.
The authors tried to determine the best treatment of intramedullary tumors nowadays. They studied a personal series of 23 intraspinal ependymomas and astrocytomas. 15 patients had a mean long term follow-up of 5 1/2 years. Among these, only patients with ependymomas were clinically cured or dramatically improved (30%). On the contrary, no patients operated on an astrocytoma received benefit of the surgical procedure. This study was compared to most of the recent series of the literature , the results of which are superimposable . Concerning an intraspinal ependymoma, a logical attitude is to proceed, under optic magnification, to the most complete excision as possible, as long as a well individualized plan of cleavage is visible. Radiotherapy is indicated only in case of incomplete excision or recidive. Concerning an astrocytoma, a total excision is almost always impossible since it is an infiltrating tumor. It appears more dangerous than advantageous to try to take off as much tumor as possible. Radiotherapy appears inefficient.