Search for poliovirus in specimens from patients with the post-polio syndrome.
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Adult↗
Biomedical subjects
J Julien
Publications and source records attributed to J Julien.
Involvement of respiratory muscles in adult-onset dystonia: a clinical and electrophysiological study.
Involvement of respiratory muscles is unusual in dystonia, but its occurrence may be underestimated either because it is not conspicuous or because it is improperly imputed to another cause. Three patients who had adult-onset dystonia and who were exhibiting respiratory problems were examined clinically and electrophysiologically. In the three patients the onset was focal-cervical in two and blepharospasm in one. The respiratory problems appeared later. The first patient had involuntary deep and loud inspirations combined with spasms of axial dystonia, the second complained of breathing arrests, and the third had deep inspirations mainly on speaking or reading aloud, thus causing broken speech. Electromyographic findings, including of the diaphragm, were quite consistent with a respiratory involvement in these three cases of dystonia. Assuming that respiratory troubles could be in the first sign of a focal dystonia, electrophysiological studies of respiratory muscles could be used to confirm this.
Administration, Oral↗
[Neurologic manifestations of Lyme disease. Apropos of 25 cases].
We studied retrospectively the cases of neurological forms of Lyme disease observed in two internal and two neurological departments from 1986 till 1993. Twenty five cases have been collected among 15 men and ten women whose mean age was 61 years. Tick bites were previously noticed in 11 cases. Erythema chronicum migrans (ECM) was mentioned in 16 cases mostly on lower limbs. The mean time between ECM and the onset of neurological symptoms was less than 1 month in 11 cases, 2 months in three cases, and 6 months in two cases. Neurological abnormalities were often associated in the same patient. Hyperalgic radiculitis (n = 16), mainly noticed in the ECM territory (n = 10) was only sensitive in six cases and associated with motor deficit in ten. Atypical polyradiculoneuritis was achieved in six cases. Clinical (n = 5) or biological (n = 22) meningitis could occur: CSF was clear with pleiocytosis (132 per mm3), mainly lymphocytic, and hyperproteinorachia (1.2 g/l) with normoglycorachia. An increase of the CSF immunoglobulins G with oligoclonal fragmentation was noticed in 11 cases. Cranial neuropathy was frequent: VII (n = 8), VI (n = 2), III, IV, VIII (n = 1). Encephalitis (with white matter demyelination) resolved partially in two cases. Diagnosis was always confirmed by Borrelia burgdorferi serology (indirect immunofluorescence) with a significant increase of the antibodies titer (n = 17) or a CSF titer > 1/4 (n = 11). Syphilitic serology was always negative. All patients were treated with parenteral beta lactamins and four with corticosteroids. Outcome was favorable in 20 patients with incomplete resolution of neurological symptoms in two patients.
Adult↗
Idiopathic CD4+ T-cell lymphocytopenia and subacute inflammatory demyelinating polyradiculoneuropathy.
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Anemia, Hemolytic↗
High-grade B-cell cerebral lymphoma in a patient with anti-myelin-associated glycoprotein IgM paraproteinemic neuropathy.
A 74-year-old woman with a sensory neuropathy and IgM M-protein monoclonal gammopathy of undetermined significance developed a fatal B-cell cerebral lymphoma. CSF protein immunofixation revealed intrathecal secretion of a paraprotein of the same heavy- and light-chain isotypes as the serum monoclonal component (IgM-lambda). Reactivation of Epstein-Barr virus was present in the lymphoma cells. Different factors may be involved in the preferential malignant development of the monoclonal B-cell clone within the CNS.
Aged↗
[Tumors of both adrenal glands. 12 consecutive cases].
OBJECTIVES: When abdominal imagery reveals bilateral adrenal tumours, diagnosis and treatment may have severe consequences leading to life-long hormone substitution. We report our series of 12 consecutive cases in order to evaluate the diagnostic procedures and propose an adapted therapeutic strategy. METHODS: Over a 5-year period we observed bilateral adrenal tumours in 12 patients (9 males and 3 females; mean age 49.2 years; range 22-67) among a population of 10,000 hypertensive subjects. Routine laboratory tests and hormonal levels were determined in all 12 patients. Abdominal computed tomography with opacification (n = 12) and magnetic resonance imagery (n = 5) was also performed. Other tests including echography and scintigraphy were performed as needed for diagnosis. All patients underwent laparotomy. RESULTS: Hormone levels were normal in 6 patients and led to the diagnosis of pheochromocytosis (n = 5) and primary hyperaldosteronism (n = 1) in the others. After laparotomy, the pathology diagnosis was bilateral pheochromocytosis (n = 5), bilateral metastasis (n = 2) and benign bilateral adenoma (n = 5). In 5 cases, hormone levels were insufficient for establishing diagnosis before laparotomy. In these cases the pathology diagnosis was benign tumour in 4 and malignant tumour in 1. Images of adrenal masses (metastases) led to the diagnosis of the primary cancer in one case. CONCLUSIONS: Search for pheochromocytosis and primary neoplasia, after eliminating adrenal myelolipoma and pseudotumours on the basis of the tomography images, should be the first exploration of fortuitously discovered bilateral adrenal masses. Exploratory puncture biopsy should be avoided and, for tumours greater than 30 mm, exploratory laparotomy is indicated. Tumours less than 30 mm should be followed echographically for six months to eliminate malignancy. Masses which increase in size should be explored surgically. Until larger series enable a standardized approach, malignancy should always be suspected in non-secreting bilateral tumours of the adrenal glands.
Adenoma↗
[Arterial pressure after transplantation].
Following organ transplantation, repeated measurements of blood pressure are an essential part of monitoring as they enable arterial hypertension, the most frequent complication in these patients, to be detected and treated at an early stage. De novo occurrence of arterial hypertension, directly due to the use of cyclosporin, is observed in more than two-thirds of these patients during the first post-transplantation year. This predominantly diastolic arterial hypertension is usually mild to moderate, but it has repercussions on graft function and requires a specific treatment appropriate to the severity of arterial hypertension and to its physiological mechanism depending on the organ transplanted. Arterial hypertension is caused by the nephrotoxicity of cyclosporin and by its intrarenal and peripheral vasoconstrictive activity. Stimulation of the renin-angiotensin system associated with stimulation of the sympathetic system and blood volume expansion play a major role in the genesis and maintenance of de novo arterial hypertension which constitutes a new model of drug-induced hypertension. Treatment of de novo arterial hypertension rests on arteriole-dilating antihypertensive drugs such as calcium channel blockers or angiotensin-converting enzyme inhibitors used as first-line therapy and sometimes combined with another drug. In case of severe and uncontrolled arterial hypertension, the other classes of antihypertensive drugs can be used as second treatment.
Heart Transplantation↗
[Mental deterioration and hypertension: uncommon manifestation of corticotropin insufficiency and functional carboxymethyl oxidase block].
We report a new case of muscle contractures associated with adrenocortical deficiency. Outstanding features were the diffusion of the contractures, rhabdomyolysis and an encephalopathy which disappeared with hormonal therapy. Endocrinological investigations revealed a functional carboxymethyl oxidase type II defect which could, in part, explain our patient's neuromuscular symptoms.
Abdominal Muscles↗
[Multifocal motor neuropathy].
We report the case of a patient with multifocal motor neuropathy. Electrophysiological studies showed typical multifocal conduction blocks, but the search for anti-GM1 antibodies was negative. This case provide the heterogeneity of this clinical entity.
Adult↗
[Treatment of arterial hypertension in patients with organ transplantation].
The onset of predominantly systolic hypertension (HT) is the most common complication following organ transplantation. The hypertension is directly linked to the immunosuppressant treatment, particularly to the nephrotoxic and intrarenal and peripheral vasoconstrictor effects of cyclosporin. Treatment is based on the primary use of arteriolar vasodilators, but must be appropriate for the organ transplanted and the degree of HT. It is often necessary to administer two or three antihypertensive drugs, which must take into account the precautions for their use and possible interactions with cyclosporin: calcium channel inhibitors inhibit the vasoconstriction induced by cyclosporin, converting enzyme inhibitors slow the compensatory glomerular hyperfiltration, diuretics are effective versus this type of HT, which is sensitive to sodium depletion, and beta-blockers are useful in hypertensive patients who have undergone kidney or liver transplants, but less commonly used in cardiac transplant patients. Centrally-acting antihypertensives are used as a second option.
Antihypertensive Agents↗
Single fiber EMG and spectral analysis of surface EMG in myotonia congenita with or without transient weakness.
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Electromyography↗
[Neurologic manifestations in Lyme disease. Apropos of 22 cases].
The main clinical and biological features of 22 cases of neurological forms of Lyme disease are reviewed. Radiculitis (n = 15), cranial nevritis (n = 7), meningitis (n = 5) and encephalitis (n = 4) are often associated. Tick bites were previously noticed in only 40% of cases; erythema chronicum migrans in 73%, "one to six months before the onset of neurological symptoms". Titers of Borrelia Burgdorferi antibodies were always above 1/256. Among 18 patients, DR W2 HLA haplotype was present in 15.
Female↗
Left ventricular mass and geometry before and after etiologic treatment in renovascular hypertension, aldosterone-producing adenoma, and pheochromocytoma.
To study left ventricular (LV) geometry in secondary hypertension and its evolution following etiologic treatment, echocardiography was performed in a total of 73 patients: 40 patients with renovascular hypertension (RVH), 21 with aldosterone-producing adenoma (APA), and 12 with pheochromocytoma (PH). Repeat echocardiography was possible in 43 of these patients, 3-24 months following curative renal revascularization or adrenal surgery. Age, sex ratio, and initial drug treatment score were comparable in the three etiologic categories, but 24-h ambulatory blood pressure and LV mass index were significantly higher in APA and RVH than in PH. End-diastolic LV volume was significantly smaller in PH than in APA and RVH. After treatment, the greatest reduction in LV mass occurred in APA (-18%, P < .05) and the lowest in PH (-5%, NS). Both patients with APA and those with PH exhibited a significant decrease in LV wall thickness, whereas LV diameter tended to decrease in APA patients and to increase in PH patients. No significant cardiac changes occurred in RVH patients after treatment. Although LV mass index and ambulatory blood pressure were correlated both before and after treatment, LV mass index changes did not correlate with changes in ambulatory blood pressure or with the known duration of hypertension. Systolic function was normal before and following etiologic treatment in the three categories. These findings suggest that, in addition to blood pressure, volume and/or humoral factors influence the pathogenesis of left ventricular hypertrophy and its reversibility.
Adenoma↗
Cyclosporine-induced stimulation of the renin-angiotensin system after liver and heart transplantation.
To analyze the status of the renin-angiotensin system in hypertensive transplant recipients on cyclosporine, we prospectively explored 21 cardiac (CTR: 52 +/- 8.2 yr) and 12 liver (LTR: 45 +/- 10 yr) transplant recipients on a normal salt diet with 19 normotensive controls in the same age range. Systolic and diastolic blood pressure was measured in the supine and standing positions. Renal function was assessed by serum creatinine values, and 24-hr urinary sodium and potassium excretion were recorded. Plasma renin activity (PRA), active renin, total renin, angiotensinogen, aldosterone, and cortisol plasma levels were simultaneously determined. Results were expressed as mean +/- SD, and between-group differences were compared using variance analysis. Supine blood pressure (+/- SD) was 158 +/- 15/103 +/- 8.4 in CTR and 155 +/- 21.4/102 +/- 11.7 mmHg in LTR. Serum creatinine was higher in CTR (159 +/- 52 mumol/L) than in LTR (117 +/- 24.7, P < 0.05) and values in both groups were above controls (83 +/- 14.1, P < 0.05). Urinary sodium excretion tended to be lower in transplant recipients (59 +/- 42 mmol/L) for CTR and 44 +/- 36.7 in LTR than in healthy controls (117 +/- 24.7 mmol/L). Supine and upright PRA values tended to be higher in hypertensive transplant recipients than in healthy volunteers, although not significantly. Supine active renin was significantly higher in CTR (47 +/- 42 pg/ml) and in LTR (44 +/- 29.8 pg/ml) than in normal subjects (17 +/- 4.8 pg/ml, P < 0.05). Total renin levels in CTR (supine: 716 +/- 357 pg/ml) and in LTR (supine: 647 +/- 365 pg/ml) were 3- to 4-fold higher than in controls (supine: 207 +/- 69 pg/ml) (P < 0.05), as were inactive renin levels (P < 0.01). Active renin was effectively correlated with PRA (P < 0.001) and with total renin (P < 0.001) in the supine and in the upright position. Plasma aldosterone was almost within the normal range in CTR and in LTR, and it did not correlate with PRA values. Plasma angiotensinogen levels were normal in LTR (1032 +/- 226 ng/ml) but were significantly lower in CTR (938 +/- 216 ng/ml, P < 0.05). Cortisol plasma levels were lower in both CTR (7 +/- 4.4 micrograms/L) and LTR (6 +/- 1.9 micrograms/L) than in healthy controls (11 +/- 4 micrograms/L, P < 0.01).(ABSTRACT TRUNCATED AT 400 WORDS)
Aldosterone↗
A transthyretin variant (alanine 49) associated with familial amyloidotic polyneuropathy in a French family.
A transthyretin mutation was discovered in a French family with familial amyloidotic polyneuropathy originally described in 1983. The syndrome is of early onset (approximate age 35 to 40) with carpal tunnel syndrome. Death is from cardiac disease. By direct genomic DNA sequencing an A-->G mutation was found in the position corresponding to the first base of transthyretin codon 49. The predicted alanine for threonine substitution in the transthyretin protein was proven by amino acid sequencing of transthyretin isolated from the plasma of an affected subject. Since the DNA mutation does not result in the creation or abolition of a restriction endonuclease recognition site, a new DNA analysis technique was used in which site directed mutagenesis is used to create an RFLP when the introduced mutation is in proximity to the natural mutation. Using a 27 nucleotide mutagenesis primer in the PCR reaction, a new Bg1I site was created on amplification of the variant allele. Using this test, termed PCR-IMRA, four affected members of the family were shown to have the mutation.
Adult↗
Late central demyelination after Fischer's syndrome: MRI studies.
The case of a patient who presented with clinical, electrophysiological, and MRI evidence of central demyelination is described. The patient had been admitted to hospital for Fischer's syndrome a few years previously. The association of these two events suggests that central and peripheral myelinopathy may be related in Fischer's syndrome.
Ataxia↗
Isolated peripheral nerve relapse masquerading as Guillain-Barré syndrome in a patient with acute lymphoblastic leukemia.
We report a 30 year old patient with acute lymphoblastic leukemia (ALL) whose leukemic relapse presented as an isolated symmetrical peripheral neuropathy with facial diplegia. Initially, this was consistent with a Guillain-Barré syndrome but the peripheral nerve biopsy revealed leukaemic infiltration. This was followed by a systemic relapse. Reports of peripheral nerve infiltration are scarce and to the best of our knowledge this is the first documented case of peripheral polyneuropathy as a presenting manifestation of ALL relapse.
Adult↗
[Limb-girdle syndrome. A study of 46 cases].
We report a series of 46 patients (32 women and 14 men) with limb-girdle syndrome. After reappraisal, another diagnosis was made in 10 of them. Becker's muscular dystrophy was the most frequent cause among men (near 50 p. 100). A Duchenne muscular dystrophy manifesting carrier was discovered among 13 reevaluated women. Among the 36 cases (29 women and 7 men) without any defined etiology, 29 were without any other known familial history. Fifteen of these women had similar clinical findings: incipient weakness in the pelvic girdle and onset of symptoms most often in the forties. In these cases serum creatine kinase activity was normal or slightly elevated, and muscle biopsy showed non-specific patterns. "Late onset muscular dystrophy in females" should be reevaluated.
Adult↗