[Meningioma of the jugular foramen with a large cervical extension]. / Meningiome du foramen jugulaire à large extension cervicale.

Meningioma rarely gives an extra cranial extension. The authors report a case of meningioma of the jugular foramen reaching the left side of the neck. Epidemiological and clinical aspects of meningiomas of the neck are presented. The pathogenesis of this ectopic meningioma is discussed. We stress the contribution of imaging to evoke the diagnosis, determine the exact extent of the tumor, guide biopsies and allow post-therapeutic surveillance. Histological diagnosis of meningiomas of the neck is difficult since it can mimic more commonly encountered neck tumours. Difficulties of surgical techniques used to manage the tumor are also reported.

[Pituitary and ovarian mass syndrome in primary hypothyroidism]. / Syndrome de masse hypophysaire et kystes ovariens dans un cas d'hypothyroïdie périphérique.

INTRODUCTION: Hyperprolactinemia and a pituitary mass syndrome can occur in patient with primary hypoyhroidism. In young women, hypothyroidism can be associated with ovarian cysts. EXEGESIS: We report a case of a 30 year-old women who was treated for an anemia. She had clinical and biological signs of primary hypothyroidism with hyperprolactinemia at the biology. Pituitary MRI showed a pituitary mass. The pelvic ultrasound examination and CT scan showed a right ovarian cyst. The subsequent thyroid hormone replacement was associated with a clinical, a biological and radiological resolution. It was a pituitary and ovarian mass syndrome secondary to primary hypothyroidism. CONCLUSION: The recognition of these associations may eliminate unnecessary surgery and lead to the choice of hormone replacement therapy.

Myocardial muscarinic receptor upregulation and normal response to isoproterenol in denervated hearts by familial amyloid polyneuropathy.

BACKGROUND: Patients with familial amyloid polyneuropathy, a rare hereditary form of amyloidosis, have progressive autonomic neuropathy. The disease usually does not induce heart failure but is associated with sudden death, conduction disturbances, and an increased risk of complications during anesthesia. Although cardiac sympathetic denervation has been clearly demonstrated, the postsynaptic status of the cardiac autonomic nervous system remains unelucidated. METHODS AND RESULTS: Twenty-one patients were studied (age, 39+/-11 years; normal coronary arteries; left ventricular ejection fraction 68+/-9%). To evaluate the density and affinity constants of myocardial muscarinic receptors, PET with (11)C-MQNB (methylquinuclidinyl benzilate), a specific hydrophilic antagonist, was used. Cardiac beta-receptor functional efficiency was studied by the heart rate (HR) response to intravenous infusion of isoproterenol (5 minutes after 2 mg of atropine, 5, 10, and 15 ng/kg per minute during 5 minutes per step). The mean muscarinic receptor density was higher in patients than in control subjects (B'(max), 35.5+/-8.9 versus 26.1+/-6.7 pmol/mL, P=0.003), without change in receptor affinity. The increase in HR after injection of atropine as well as of MQNB was lower in patients compared with control subjects despite a similar basal HR (DeltaHR after atropine, 11+/-21% versus 62+/-17%; P<0.001), consistent with parasympathetic denervation. Incremental infusion of isoproterenol induced a similar increase in HR in patients and control subjects. CONCLUSIONS: Cardiac autonomic denervation in familial amyloid polyneuropathy results in an upregulation of myocardial muscarinic receptors but without change in cardiac beta-receptor responsiveness to catecholamines.

Cardiac sympathetic denervation in familial amyloid polyneuropathy assessed by iodine-123 metaiodobenzylguanidine scintigraphy and heart rate variability.

Familial amyloid polyneuropathy (FAP) is a rare and severe hereditary form of amyloidosis, due to nervous deposits of a genetic variant transthyretin produced by the liver and characterized by both sensorimotor and autonomic neuropathy. Left ventricular systolic dysfunction is rare, but conduction disturbances and sudden deaths can occur. The neurological status of the heart has not been elucidated, and an alteration of the sympathetic nerves may be involved. We studied 17 patients (42+/-12 years) before liver transplantation by iodine-123 metaiodobenzylguanidine (MIBG) scintigraphy, heart rate variability analysis, coronary angiography, radionuclide ventriculography, rest thallium single-photon emission tomography (SPET) and echocardiography. Coronary arteries, left ventricular systolic function and rest thallium SPET were normal in all patients. Only mild evidence of amyloid infiltration was found at echocardiographic examination. Cardiac MIBG uptake was dramatically decreased in patients compared with age-matched control subjects (heart-to-mediastinum activity ratio at 4 h: 1.36+/-0.26 versus 1.98+/-0.35, P<0.001), while there was no difference in MIBG washout rate. Heart rate variability analysis showed a considerable scatter of values, with high values in four patients despite cardiac sympathetic denervation as assessed by MIBG imaging. The clinical severity of the polyneuropathy correlated with MIBG uptake at 4 h but not with the heart rate variability indices. Cardiac MIBG uptake and the heart rate variability indices did not differ according to the presence or absence of conduction disturbances. Patients with FAP have sympathetic cardiac denervation as assessed by MIBG imaging despite a preserved left ventricular systolic function and cardiac perfusion, without correlation with conduction disturbances. Results of the heart rate variability analysis were more variable and this technique does not seem to be the best way to evaluate the extent of cardiac sympathetic denervation in FAP patients.

[Diagnosis of ventricular tachycardia by electrocardiography]. / Diagnostic électrocardiographique des tachycardies ventriculaires.

In monomorphic wide QRS complex tachycardia, it is important to differentiate ventricular tachycardia from supraventricular tachycardia with aberration or preexcitation both from the prognostic and therapeutic view points. Atrioventricular dissociation with fusion complexes allows diagnosis of ventricular tachycardia but the negative predictive value of these criteria is low. Extreme QRS axis deviation, concordant morphological criteria in leads V1-V2 and V6 and analysis of the RS complexes in the precordial leads, nearly always enable supraventricular tachycardia with aberration. The distinction with other causes of wide QRS complex tachycardias (supraventricular tachycardia with preexcitation or with non-systematized intraventricular conduction defects) is much more difficult in the absence of a reference recording and depends more on the clinical context than ECG analysis.