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Biomedical subjects

G Monnier

Publications and source records attributed to G Monnier.

At least 37 records · Page 2Linked to original sources

Exercise test in muscle channelopathies and other muscle disorders.

We studied the percentage change in compound muscle action potential (CMAP) amplitude and area during and after a 5-min maximal contraction of the muscle. The exercise test (ET) was performed on 64 patients with different muscle disorders and on 46 normal controls. The range of normal ET values was defined as the mean + 2 SD of the control values. The mean sensitivity of the test was 63% in the whole group with ion channel muscle disorders, the highest sensitivity being seen in primary periodic paralysis (81%) and the lowest in chloride channelopathies (17%). In thyrotoxic periodic paralysis, the ET was abnormal in the three of the four patients studied. In patients with myotonic dystrophy, a smaller than normal increase in CMAP amplitude occurred during and after exercise, whereas in proximal myotonic myopathy a normal initial increase in CMAP amplitude was followed by an abnormal decrement. We conclude that the ET can be of use in confirming abnormal muscle membrane excitability in patients with calcium and sodium channelopathies and thyrotoxic periodic paralysis. In chloride channelopathy, the test may also be abnormal, but shows no, or only a small, increase in amplitude or area in the immediate postexercise period. The test may also be abnormal in proximal myotonic myopathy, but is normal in myotonic dystrophy.

Action Potentials↗

[A new case of pericardial thymic cyst].

The thymic cysts are benign tumours of the thymo-pharyngeal canal, usually located in the cervico-mediastinal region. The authors report the case of a large thymic cyst with an ectopic right paracardiac location compressing the right heart chambers. It was a chance finding on chest X-ray of an asymptomatic 21 year old man. Though suggestive of a pericardial cyst in view of its position, curative surgical ablation allowed confirmation of the diagnosis at anatomo-pathological examination.

Adult↗

[Paraganglioma associated with renal artery stenosis: unusual cause of secondary arterial hypertension. A case report and review of the literature].

Secreting paraganglioma is a rare cause of secondary HT and corresponds to an extra-adrenal phaeochromocytoma. This case reports the consecutive coexistence, with a free interval of 10 years, of dysplastic renovascular HT and abdominal paranganglioma in the organ of Zuckerkandl. This is a rare association which requires investigation of a common genetic predisposition (neural crest disease) and emphasizes the importance of long-term surveillance of these patients.

Adult↗

[Isolated primary streptococcal tricuspid endocarditis. A case report].

The authors report a new case of primitive isolated endocarditis of the tricuspid valve. The microorganism responsible, Streptococcus sanguis, is unusual in its localization and in this environment. The uncomplicated evolution poses the problem of deciding what action should be taken vis-à-vis the persistence of an important isolated vegetation after effective antibiotic treatment. Therapeutic abstention was chosen rather than surgical intervention in light of the literature. A spontaneous pulmonary embolic migration of the vegetation was effectuated 3 weeks after termination of the antibiotic treatment without pulmonary or hemodynamic complications.

Endocarditis, Bacterial↗

[Chronic occlusion of the left coronary artery].

Chronic occlusion of the left main coronary artery is rare with less than 100 reported cases. the treatment is essentially surgical but medical management has been proposed. This case report illustrates the prognostic benefit of surgical management in view of the risk of secondary occlusion of the proximal right coronary artery.

Chronic Disease↗

[Acute arterial hypertension caused by dissecting aneurysm of the renal artery].

BACKGROUND: Aneurysm of the renal artery is an uncommon discovery at arteriography performed as part of a hypertension work-up. CASE REPORTS: We observed acute hypertension following dissection of a renal artery aneurysm which led to circulating channel stenosis. After surgical correction of the lesion, arterial pressures returned to normal. DISCUSSION: Most cases of renal artery aneurysm do not cause hypertension. In such cases, the high blood pressure is idiopathic or related to fibrodysplastic stenosis of the renal artery often associated with aneurysm formation. In rare cases with obstructive complications alone an aneurysm may lead to acute hypertension either after dissection as in our case or after thrombus formation. Surgery is generally required.

Acute Disease↗

[Unilateral asterixis and focal brain lesions. 12 cases].

Asterixis is a involuntary movement with spontaneous interruptions and intermittent muscle tone. It occurs during posture maintenance. It is usually bilateral and, in this case, is observed in metabolic encephalopathy. Unilateral asterixis is more uncommon. We report 12 cases which occurred in patients with focal brain lesions. In all cases asterixis involved the upper limb and the lower limb in only 2 cases. Asterixis was transient, discrete and always associated with other neurological signs. The causal lesions (7 infracts, 2 haematomas, 2 abscesses, 1 meningioma) were unique in 7 cases and multiple in 5. Asterixis was always contralateral to the unilateral lesions. Lesions mainly involved the thalamus (7 cases) and other structures (lenticular nucleus, frontal lobe, internal capsule, precentral regions and cerebellum). The frequency of thalamic involvement suggested dysfunction of the thalamo-cortical loop.

Aged↗

[Silent exercise-induced enzymatic myopathies at rest in adults. A cause of confusion with fibromyalgia].

Exercise-induced enzymatic myopathies include carnitine palmityl transferase deficiency and, among muscular glycogenoses, Mac Ardle's and Tarui diseases. These diseases are usually recognized when exercise-induced myalgias, myoglobinuria and raised creatinine kinase (CK) levels are present. However, myoglobinuria may be absent in 10 to 50 percent of the cases, and CK levels are often normal at rest; thus, the diagnosis is often delayed for several years, with a risk of acute renal failure in 10 to 30 percent of the patients. We report 6 cases of exercise-induced enzymatic myopathies with normal CK levels and with electromyographic studies at rest. The main clinical features of these cases and those of similar conditions reported in the literature are male sex, onset of the disease before the age of 15 years, episodes of severe exercise-induced myalgias, cramps and muscle weakness and myogenic hyperuricaemia at rest in muscular glycogenosis.

Adolescent↗

[Congenital myotonic dystrophy. Diagnostic difficulties].

Congenital myotonic dystrophy is the neonatal form of Steinert myotonia. It is particularly severe when the mother carries the gene. The diagnosis is made after finding the myotonia in the mother. Prenatal diagnosis can be made by researching the Steinert's disease gene by fetal DNA analysis.

Adult↗

[Suprascapular nerve entrapment at the spinoglenoid notch].

The suprascapular nerve passes through the spinoglenoid notch with a risk of entrapment. This results in distal nerve lesion characterized by isolated paralysis of the infraspinatus muscle and, most often, by shoulder pain. We report 7 clinical and electromyographical cases of pure infraspinatus muscle paralysis. The value of the electrodiagnosis, which demonstrated prolonged suprascapular distal nerve latencies (over 5 milliseconds) in the infraspinatus muscle affected while latencies were normal in the supraspinatus muscle, is emphasized. Mechanical factors were associated with paralysis in 5 cases. Compressive synovial cysts were found in 2 patients operated upon. Surgical enlargement of the spinoglenoid notch regularly and rapidly relieves pain and sometimes helps in recovery of the infraspinatus muscle.

Adolescent↗

[Haemophilus influenzae purulent pericarditis].

A case of hemophilus influenzae type B pericarditis in a 2-yr-old infant is reported. The authors emphasize the interest of cardiac ultrasound and pulse Doppler for diagnosis, the evaluation of hemodynamic consequences and follow-up.

Child, Preschool↗

[Carpal tunnel syndrome before 20 years of age. A report of 7 cases].

Seven cases of carpal tunnel syndrome before 20 years old are described: 4 were associated with an increase in the patient's athletic activities. In five cases, no etiology was found, marked median nerve stenosis was observed in three cases and hypertrophy of the median nerve was observed in two cases. Flexor retinaculum was thickened in four cases.

Adolescent↗

Polyneuropathy: an unusual extraintestinal manifestation of Crohn's disease.

Among the extraintestinal complications of Crohn's disease, neuropathy seems to be rare, and is only exceptionally reported in the literature. The authors present the report of a patient followed over a 12-year period who developed polyneuropathy with a parallel course to Crohn's disease. There was no evidence of any other underlying condition. On one occasion, the patient developed a cutaneous vasculitis with fibrinoid deposits in the vessels. The search for circulating immune complexes was positive. The authors demonstrated increased intestinal permeability using the 51chromium-labeled ethylenediaminetetra-acetate (51Cr-EDTA) test. Ultrastructural study of a biopsy of the superficial peroneal nerve disclosed a marked denervation process. Therapeutic plasmapheresis were performed, improving the neurologic symptoms. Vasculitis with circulating immune complexes is suggested as a possible mechanism in view of the occurrence of cutaneous vasculitis, increased intestinal permeability, and plasma exchange efficiency.

Biopsy↗

[Denny-Brown sensory neuropathy disclosing a bilateral ovarian adenocarcinoma].

The authors report the case of a 76 year-old woman examined because of occurrence of a paraneoplastic sensory neuropathy. Three months later, she developed a left inguinal adenopathy, metastasis of a bilateral ovarian adenocarcinoma. This case of particular interest in view of the way the neoplasm was discovered. The sensory neuropathy was previously reported to be associated with an ovarian cancer in only one case of the literature. Besides the tumor masse only limited to a left inguinal adenopathy, in this case emphasizes the ability of these rare metastasis. The ovarian cancer-associated antigen CA 125 is particularly useful for the diagnosis when high levels are found. In the absence of a pelvic tumor masse, the paraneoplastic sensory neuropathy, as well as the high levels of CA 125, have resulted in an exploratory laparotomy with extemporaneous examination of the ovaries, enabling to make the diagnosis of bilateral ovarian adenocarcinoma.

Adenocarcinoma↗

[Carpal tunnel syndrome in childhood. Apropos of a familial and idiopathic case].

The authors report an uncommon' case of carpal tunnel syndrome in a ten year old girl. Symptoms are typical with noctural paresthesias. The nerve conductor studies show a important compression with thenar atrophy. In consideration of the importance of nocturnal pain and amyotrophy an operation was decided. At operation, the median nerve is found constricted under tickened transverse carpal ligament. At the wrist, the volume of nerve is normal, there are not anomalous muscles or inflammatory tenosynovitis. In fact, the appearance of median nerve is identical to the cases of carpal tunnel syndrome in adult. After surgery symptomsare abolished. The mother of the young girl, 37 year old, also present a bilateral carpal tunnel syndrome with onset at the age of 10 years. There is a important thenar atrophy. At operation, the nerve is very constricted with a tickened transverse carpal ligament; no other anomaly is noted. A study of literature complete these observations.

Adult↗