PubMed Health⌕ Search

Biomedical subjects

J D Degos

Publications and source records attributed to J D Degos.

At least 55 records · Page 3Linked to original sources
PubMed1995

Diphtheritic neuropathy.

A 29-year-old Haitian man had a sore throat with a marked tonsillar exudate followed by the onset of increasingly blurred vision with swallowing difficulties and, then, a diffuse acute demyelinating neuropathy. Cerebrospinal fluid showed moderate pleiocytosis and elevated protein content. Serological testing for diphtheria, but not for tetanus and poliomyelitis, was markedly positive. Other tests including HIV1 and 2, HTLV1 and Lyme disease were negative. Diphtheritic neuropathy should be considered in the differential diagnosis of Guillain-Barré syndrome in nonvaccinated patients.

Adult↗
PubMed1995

Methylprednisolone infusion during acute exacerbation of MS: plasma and CSF concentrations.

Plasma and CSF concentrations of methylprednisolone (MP), after a high-dose intravenous administration (1,500 mg) prescribed to patients presenting acute exacerbation of multiple sclerosis, were measured. A plasma pharmacokinetic study with 1 CSF sample was performed in 9 patients and 4 other patients had a single plasma sample at the time of lumbar puncture. MP plasma kinetics were similar between patients. Pharmacokinetic parameters were very close to previous published values obtained in normal volunteers or patients with rheumatoid disease. Passage of MP into the CSF was delayed since mean plasma/CSF ratio dropped dramatically from 150 at the 2nd h to 25 at the 3rd h after starting infusion. CSF concentrations of MP were high with a mean reaching 522 micrograms/l at the 6th h. The high levels of CSF concentrations suggest that MP may have powerful pharmacological effects within the CNS. No correlation was found between CSF concentration and the clinical response to steroid therapy. The issues related to previous data on clinical, biological and radiological effects of high-dose intravenous MP are discussed in the light of our results.

Adult↗
PubMed1995

[Extracerebral metastases of a glioblastoma, in the absence of surgery].

A 50 y.o. male presented with a right parietal tumor which was a glioblastoma on stereotactic biopsy. He was treated by radiation and steroids, with clinical improvement. Four months later, he presented with a left preauricular mass and cervical lymphadenopathy. CT scan showed destruction of the left mastoid and filling of the left tympanic cavity. One month later, he suffered progressive dyspnea. Chest X ray showed a mediastinal mass on the right side and numerous bilateral interstitial opacities in the lungs. A bronchial biopsy was inconclusive. His general condition worsened, and he died. Postmortem showed continuous neoplastic infiltration of the left part of the base of skull, extending into the neck. Numerous metastases were present in mediastinal lymph nodes, lung parenchyma, pleura and pleural aspect of the diaphragm. There were no subdiaphragmatic metastases. Neuropathological examination confirmed a poorly differentiated highly malignant glioblastoma with severe necrosis involving the internal part of the parietal lobe extending to the dura mater of the convexity and falx cerebri with invasion of the superior longitudinal sinus which was entirely occluded. The biopsy scar was not infiltrated. Visceral tumors were morphologically identical to the brain tumor. They were strongly GFAP positive and cytokeratin negative. Extraneural metastases of glioblastoma in the absence of surgery are uncommon in adults. Involvement of the dura mater and/or superior longitudinal sinus is an almost constant feature. In our case, this may have led to invasion of the base of skull and secondary regional, lymphatic, and hematogenous spread.

Brain Neoplasms↗
PubMed1994

Elevated levels of interleukin-1 beta (IL-1 beta) and IL-6 in serum and increased production of IL-1 beta mRNA in lymph nodes of patients with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes (POEMS) syndrome.

To evaluate a possible implication of cytokines in the pathogenesis of polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes (POEMS) syndrome, we studied five consecutive patients with this condition, of which four had sclerotic bone lesions and four had multicentric Castleman's disease. Interleukin-1 beta (IL-1 beta) and IL-6 serum levels were determined in these patients (13 serum samples) and in patients with multiple myeloma (5) and Waldenström's macroglobulinemia (5). In situ hybridization of the relevant mRNAs was performed on lymph node specimens of two patients with POEMS syndrome who had Castleman's disease. Elevated serum levels of IL-1 beta (13/13 samples), and IL-6 (7/13 samples) were found in patients with POEMS syndrome. In the other patients, serum IL-1 beta was undetectable or slightly increased and IL-6 was elevated in a single patient with Waldenström's macroglobulinemia. Abundant IL-1 beta mRNA-producing cells were present in interfollicular spaces in the two tested patients, while IL-6 mRNA-producing cells were rare. We conclude that IL-1 beta and IL-6 serum levels may be chronically elevated in patients with POEMS syndrome, and that lymph node may be one site of IL-1 beta overproduction. These results are in keeping with the hypothesis that cytokines mediate systemic manifestations of POEMS syndrome.

Adult↗
PubMed1994

Early weight loss and high serum tumor necrosis factor-alpha levels in polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes syndrome.

We studied tumor necrosis factor-alpha (TNF-alpha) levels in serial serum samples from 3 consecutive patients who had a complete form of POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes) syndrome and early weight loss. Serum TNF-alpha levels were compared with those of 10 patients with multiple myeloma (5) or Waldenström's macroglobulinemia (5). Elevated serum levels of TNF-alpha were found in the patients with POEMS syndrome (two- to eightfold increase in 10 of 11 samples) and not in those with other malignant plasma cell dyscrasias. These results are in keeping with the hypothesis of a role for nonimmunoglobulinic mediators in the pathogenesis of the POEMS syndrome, and are consistent with previous reports of TNF-alpha overproduction in inflammatory demyelinating neuropathies and cachectic states.

Adult↗
PubMed1994

Creutzfeldt-Jakob disease and cerebral amyloid angiopathy.

An 83-year-old female with no personal or familial neurological history developed progressive gait and speech disturbance and left motor deficit. She suffered intractable seizures and died 3 months after the onset of neurological signs. Neuropathology showed severe spongiosis and gliosis in the cortex and basal ganglia, and diffuse cerebral amyloid angiopathy. Immunostaining for prion protein (PrP) showed intense PrP positivity in areas of confluent spongiosis and some granular staining in astrocytes. The cortical vessel walls stained positively for beta/A4 amyloid but not for PrP amyloid. Both types of amyloid were only observed in pericapillary parenchyma, in areas with severe spongiosis. There were only a few tangles and neuritic plaques in the temporal cortex; amyloid plaques were not present either by silver stains or immunostains. There was neither arteriopathic leukoencephalopathy nor cerebral hemorrhage. Immunoblot analysis of brain extracts revealed an abnormal proteinase K-resistant isoform of PrP. Association of Creutzfeldt-Jakob disease and Cerebral amyloid angiopathy in the absence of Alzheimer changes in unusual. The association of PrP and beta/A4 amyloid deposits could have been fortuitous in an 83-year-old patient. An etiopathogenic relationship between beta/A4 amyloid deposition and PrP accumulation may also be considered.

Aged↗
PubMed1994

Selective deficit of visual size perception: two cases of hemimicropsia.

Hemimicropsia is a rare disorder of visual perception characterized by an apparent reduction of the size of objects when presented in one hemifield. We report two cases of hemimicropsia resulting from focal brain lesions. The first patient was an art teacher and could accurately depict his abnormal visual perception. He subsequently died and his brain was examined post mortem. In the second patient, micropsia was assessed by a quantified size comparison task. The size of a given object is normally perceived as constant across any spatial position. Hemimicropsia may thus be considered a limited violation of the size constancy principle. Behavioural and anatomical data are discussed in relation to the neural basis of visual object perception in humans.

Autopsy↗
PubMed1994

Rest tremor and extrapyramidal symptoms after midbrain haemorrhage: clinical and 18F-dopa PET evaluation.

A 25 year old man had an acute subarachnoid haemorrhage due to the rupture of a right peduncular subthalamic arteriovenous malformation. Seven months later he developed a left rest tremor associated with mild bilateral extrapyramidal symptoms and responsive to levodopa treatment. Surface EMG recording showed synchronous activity of agonist and antagonist muscles in the left limbs. A PET 18F-dopa study showed a large decrease of the Ki value in the right striatum. One year after the stroke a persistent postural component developed in the tremor.

Adult↗
PubMed1993

Thalamic stimulation and proximal tremor. A specific target in the nucleus ventrointermedius thalami.

The severe proximal cerebellar postural tremor (also called action or intention or hyperkinetic tremor) is barely influenced by thalamotomy or stimulation of the thalamus at the classic target in the lower part of the nucleus ventrointermedius thalami (VIM). In four patients with a severe postural distal and proximal tremor, an electrode fitted with four points of contact was introduced within the entire height of the VIM. In each patient, stimulation of the lower part of the VIM was most effective in the distal component of the tremor, whereas its proximal component was specifically reduced by stimulation of its upper part. These results indicate that (1) proximal postural tremors can be as much affected by stimulation of the VIM as distal tremors, (2) there is a somatotopy in the VIM that is similar to that in the nucleus ventralis posterolateralis thalami, and (3) it is possible with this technique to adjust the VIM stimulation site so as to obtain the maximum efficacy according to the locale of the tremor.

Adolescent↗
PubMed1993

Crossed aphasia with visceral situs inversus.

A patient presenting the association of anomalous cerebral dominance for language, as revealed by crossed aphasia, and partial visceral situs inversus is described. Although it cannot be excluded that the two abnormalities coincided by chance, it is suggested that they both resulted from a single aberration of development.

Aged↗
PubMed1993

Lyme disease presenting as a stroke in the vertebrobasilar territory: MRI.

A 28-year-old female farmer, without vascular risk factors, developed a limited infarct of the pons, associated with a lymphocytic cerebrospinal fluid (CSF) pleocytosis. Titres of specific antibodies against Borrelia burgdorferi were high in serum and CSF. MRI confirmed an infarct in the territory of the medial pontine arteries, but angiography showed no evidence of cerebral angiopathy. Antibiotic therapy rapidly led to a return to normal of CSF cytology and serology. We suggest that Lyme disease is a possible cause of cerebral ischaemia.

Adult↗