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Biomedical subjects

M Pagès

Publications and source records attributed to M Pagès.

At least 19 recordsLinked to original sources

[Late onset forms of myasthenia gravis. Comparison with early-onset myasthenia gravis].

INTRODUCTION: The incidence of myasthenia gravis appears to be increasing in elderly but few studies have been devoted to late onset myasthenia gravis. PATIENTS AND METHODS: We retrospectively compared myasthenic patients with an age at onset above or below 35 years which were observed in two departments of Neurology from 1980 to 2002. RESULTS: 81 cases were included, 28 of which were late onset myasthenia gravis. The two populations were similar in terms of sex-ratio, clinical symptoms, course of the disease and therapeutic response. There was a trend for older patients to present more frequently at onset with dysphagia and axial or proximal involvement, and to have extra-ocular symptoms more quickly. Antibodies against acetylcholine receptor and striated muscle were statistically more frequent in elder patients. CONCLUSIONS: A late onset is not a factor of poor prognosis in myasthenia gravis and older patients must be treated in the same way than younger ones.

Adolescent↗

[Amyloid cardiomyopathy: A rare cause of cerebral embolism].

INTRODUCTION: We report a case of amyloid cardiopathy revealed by a cerebral embolism. CASE REPORT: A 55-year-old patient was admitted with a right hemiplegia and aphasia due to an infarction in the middle cerebral artery territory. Echocardiography was suggestive of an amyloid cardiopathy, and an IgG lambda multiple myeloma with renal insufficiency was discovered. The patient died suddenly 4 months later after chemotherapy was initiated. CONCLUSION: Embolic complications are rare and late in cardiac amyloidosis. The diagnosis may be suspected by echocardiography.

Amyloid↗

[Adult onset nemaline myopathy revealed by respiratory insufficiency].

INTRODUCTION: We report a case of nemaline myopathy revealed in adulthood by a respiratory insufficiency. CASE REPORT: A 26-year-old patient, without past history, was admitted with respiratory and right cardiac insufficiency which appeared in a few days. There was a severe restrictive lung impairment with nocturnal hypoventilation. Minor skeletal abnormalities and areflexia suggested a congenital myopathy. Muscle biopsy revealed a nemaline myopathy. CONCLUSION: Respiratory insufficiency is common in nemaline myopathy with infancy or childhood onset, but very rare in adults. It may be explained by multiple mechanisms.

Adult↗

[Relapsing ophthalmoplegia as a presenting symptom of orbital myositis].

INTRODUCTION: We report a case of orbital myositis revealed by a relapsing and initially painless ophthalmoplegia. CASE REPORT: A 50-year-old patient suddenly presented with an isolated palsy of the right lateral rectus muscle which recovered in 3 weeks after a short-lasting corticosteroid therapy but relapsed. Cerebral and orbital imaging were normal. Orbital pain with a fluctuating course occurred 6 months later. Two years after the clinical onset, orbital CT scan revealed a fusiform hypertrophy of the right lateral rectus muscle which was enhanced by contrast. After corticosteroid treatment, the symptoms improved and CT scan became normal. CONCLUSION: Orbital myositis may not be ruled out in case of painless ophthalmoplegia and systematic and repeated imaging is necessary.

Adrenal Cortex Hormones↗

[Cerebral angiitis caused by periarteritis nodosa].

INTRODUCTION: We report a case of periarteritis nodosa revealed by a cerebral angiitis which recovered under treatment. OBSERVATION: A 52-year-old patient suddenly presented with a left sensory syndrome and a fluctuating aphasia due to ischemia involving both parietal lobes. The diagnosis of periarteritis nodosa was based on the following criteria: severe loss of weight, renal insufficiency, hypertension, angiography suggesting an arteritis. Instead of an ileo-cecal perforation, the patient recovered under corticosteroid and immunosuppressive therapy. CONCLUSIONS: Stroke in periarteritis nodosa may occur early be and associated with a good outcome.

Aphasia↗

[Non-Hodgkin's malignant lymphoma of the peripheral nervous system: clinicopathological correlations in ten patients].

INTRODUCTION: Identifying tumor infiltration or compression in patients with non-Hodgkin's malignant lymphoma presenting peripheral neuropathy can be a difficult task. METHODS: We collected a series of patients with peripheral neuropathy with demonstrated lymphomatous infiltration or compression managed between October 1977 and October 2001 to search for clinico-pathological correlations. RESULTS: Ten cases were reviewed. Neurological manifestations were the inaugural symptom of the disease in 7 patients. Clinical presentations included 5 focal (3 cranial nerve palsies, 2 brachial radiculopathies) and 5 diffuse neuropathies (3 polyradiculoneuropathies, 1 polyneuropathy and 1 mononeuritis multiplex). The mechanisms of peripheral nerve involvement were classified into lymphomatous meningoradiculitis (5 cases), involvement of cranial nerves or spinal roots in their extraneuraxial course (3 cases) and infiltration of distal peripheral nerves (2 cases). Four long lasting survivals after treatment were observed. CONCLUSIONS: Prognosis depends much more on the haematological disease than on the neurological symptoms or tumor location.

Adult↗

[Ataxic sensory neuropathy and Lyme disease].

INTRODUCTION: The clinical spectrum of peripheral neuropathies in Lyme disease is very wide. We report a case which was revealed by an ataxic sensory neuropathy. OBSERVATION: A 77-year-old patient presented with a subacute ataxic sensory neuropathy which occurred 2 weeks after a skin lesion involving the right lower limb. He fully recovered after specific antibiotic treatment. EMG was suggestive of a predominantly axonal neuropathy. Diagnosis of Lyme disease was assessed by progressive elevation of serum antibodies, demonstration of a lymphocytic meningitis and intrathecal synthesis of antibodies. CONCLUSION: Lyme disease may be added to the list of diseases which may induce subacute sensory neuropathies.

Aged↗

[Intracranial haemorrhage associated with phaeochromocytoma].

INTRODUCTION: Pheochromocytoma is rarely disclosed by intracranial hemorrhage. We report two cases. OBSERVATION: The first 26-year-old patient developed subarachnoid hemorrhage due to a ruptured aneurysm of the middle cerebral artery. The second patient, aged 44 years, had a temporal hematoma. Diagnosis was suggested in both patients by hypertension and elevated urinary catecholamines and confirmed by imaging and MIBG scintigraphy. Adrenal gland tumors, on both glands in the first patient and on the right gland in the second were successfully removed; cranial hypertension totally regressed. Von Hippel Lindau disease was diagnosed by molecular genetics in the first patient. Paroxysmal hypertension could explain the brain hemorrhage in the first patient and may have favored aneurysmal rupture in the second. CONCLUSION: The relationships between pheochromocytoma and cerebral aneurysm are discussed.

Adrenal Gland Neoplasms↗

[Facial palsy and central nervous system infection with varicella virus following adult chickenpox].

INTRODUCTION: VZV virus-related peripheral neuropathies usually occur after shingles in adults and more rarely after chickenpox in childhood. CASE REPORT: A 54-year-old patient presented with a right VIIth nerve palsy following a chickenpox rash and recovered after antiviral treatment. CSF analysis revealed lymphocytic meningitis and the virus was identified by PCR. CONCLUSIONS: Although previous chickenpox was not found in the patient's past history, the probability of reinfection is likely. The virus can be assumed to affect the nervous system directly; the axonal or demyelinating mechanism of the neuropathy may be discussed.

Acyclovir↗

[Hypertensive encephalopathy as revealing symptom of Takayasu's arteritis].

A 29-year-old patient presented with Takayasu's arteritis which was revealed by heart failure, epilepsy, right hemiparesis and fever. Transient abnormalities of MRI and CSF (raised protein and cell content) were initially observed. The hypothesis of a hypertensive encephalopathy is suggested.

Acyclovir↗

[Pituitary tumour as a presenting symptom of a systemic lymphoma].

Pituitary non Hodgkin malignant lymphomas are rare. We report a case of large cell systemic lymphoma with pituitary and meningeal localizations in a 40-year-old patient. The clinical picture was panhypopituitarism with MRI appearance of pituitary adenoma and VII and VIII cranial nerves involvement. Diagnosis was made by identification of lymphomatous cells in CSF. The patient deceased in less than one year despite chemotherapy.

Adult↗

[Hypokalaemic paralysis as a presentation of adrenal tumor].

A 24-year-old patient presented with flaccid quadriplegia due to severe hypokaliemia, initially presumed to have been induced by glycyrrhizin. Persistence of low potassium levels and hypertension led to the diagnosis of primary hyperaldosteronism related to an adrenal cortical tumor. After surgery, the patient recovered from hypertension and hypokaliemia.

Adrenal Cortex Neoplasms↗

[Cerebral hemorrhage due to a ruptured mycotic aneurysm. Two cases].

INTRODUCTION: Cerebral hemorrhages due to rupture of mycotic aneurysms are rare but severe complications of infective endocarditis. We report two cases with a good outcome. EXEGESIS: The first patient presented with a parieto-occipital hematoma which occurred in the course of a relapsing infective endocarditis due to Streptococcus mitis. She fully recovered after neurosurgical treatment. In the second case, a right frontal hematoma revealed two mycotic aneurysms and an infective endocarditis due to Streptococcus gordonii. Motor weakness partially recovered after antibiotic therapy and angiography demonstrated complete resolution of aneurysms. CONCLUSION: Ruptured mycotic aneurysms are poor prognosis factors in infective endocarditis. Adapted antibiotic therapy is the first-intent treatment. Neurosurgery is indicated when hematomas are poorly tolerated and in cases requiring anticoagulant therapy.

Adult↗

[Continuous nodal reciprocal rhythm and Steinert's disease: treatment by double chamber stimulation. A case report].

We report a case of continuous supraventricular tachycardia in a patient affected by Steinert's myotonic dystrophy. The investigation of this tachycardia showed that there was a "slow-fast" common nodal re-entry, rendered continuous by the existence of significant conduction defects in the fast pathway and the slow anterograde pathway. Implantation of a double chamber cardiac stimulator, necessary for conduction defects present in the basal state in this patient allowed, with the evolution of the conduction defects, the complete eradication of reciprocal rhythm entry, without resorting to ablation.

Electroencephalography↗