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Biomedical subjects

J M López-Domínguez

Publications and source records attributed to J M López-Domínguez.

12 recordsLinked to original sources
PubMed1998

[Patient with Hansen disease and lepromatous reaction with predominant neural involvement].

We describe a patient with a diagnosis of Hansen's disease borderline type, presenting as cutaneous lesions and silent multineuritis. Samples of nasal mucus, earlobe and cutaneous lesions were positive for acid-fast bacilli. He was given treatment with rifampin, dapsone and clofazimine. Five years later, he developed fever, poliarthritis, orchitis and hepatic involvement. Searching for acid-fast bacilli in many cutaneous and mucosal locations was fruitfulness. Because of clinical suspicion of erythema nodosum leprosum, he was treated with steroids with improvement of his clinical picture, but subsequently he developed multineuritis with many sensitive symptoms. A high number of bacilli was seen in nerve biopsy. We comment on atypical features of clinical evolution and erythema nodosum leprosum, and emphasize the significance of large number of bacilli into peripheral nerve in contrast with their absence at other levels.

Biopsy↗
PubMed1997

[Diagnosis of centronuclear myopathy in adults].

INTRODUCTION: Centro-nuclear myopathy is a congenital myopathy characterized by the presence of central nuclei on muscle biopsy. Three clinical forms have been distinguished. Classification depends on the type inherited, age of onset and degree of muscle involvement. CLINICAL CASE: We describe the case of a female patient in whom the diagnosis of centro-nuclear myopathy was made at the age of 53. The patient had not been studied previously, but was sent to us by the Department of Anaesthesia. The clinical features had first appeared in infancy. There was no family history of this disorder. Apparently this was a sporadic case. CONCLUSIONS: In the differential diagnosis of adult patients with girdle paresthesias centro-nuclear myopathy should be included. This unusual muscle disorder may be need to be considered if anaesthesia is required.

Diagnosis, Differential↗
PubMed1996

[Bilateral hypoacusia and basilar dolichoectasia].

INTRODUCTION: The clinical findings of basilar dolichoectasia (DB) are caused by compression of the cranial nerves, vertebrobasilar ischaemia, hydrocephaly and compression of the brainstem. Hypoacusia as an isolated finding in DB is very rare. CLINICAL CASE: We describe the case of a 48-year-old hypertensive man with bilateral hypoacusia as an isolated finding in DB. Diagnosis was made on MR, and arteriography showed changes characteristic of an anomalous blood vessel. CONCLUSIONS: In patients with hypoacusia of uncertain aetiology, specially if they are also hypertensive, one should rule out the possibility of DB using cerebral magnetic resonance or angioresonance.

Audiometry, Pure-Tone↗
PubMedDate not supplied

[Lumbosacral plexopathy as a form of presentation of an aneurysm of the iliac artery].

INTRODUCTION: The etiology of lumbosacral plexopathy is often due to compression. One of the less common causes of this is aneurysm of the iliac artery. However, 13% of the patients with this disorder initially have symptoms of plexus irritation or deficit. CLINICAL CASE: We describe the case of a 42 year-old-man, with no previous medical history, who complained of right-sided sciatica for the previous three months. On examination there were clinical signs of a lesion of the right lumbosacral plexus. On CT of the pelvis and MR of the lumbar spine there were images compatible with an aneurysm of the right iliac artery. This diagnosis was confirmed on arteriography. Treatment was surgical (aneurysmography and right iliofemoral by-pass). The pain disappeared and the motor deficit improved considerably. CONCLUSION: When a patient presents with lumbosacral plexopathy, an iliac aneurysm should be considered as a possible etiological factor. In such cases early diagnosis is essential, since surgical treatment will usually resolve the clinical problem.

Adult↗
PubMedDate not supplied

[Uremic myopathy].

INTRODUCTION: The neurological features of chronic renal failure are very varied. Uremic myopathy is a controversial condition. In most cases it is related to the secondary hyperparathyroidism which these patients develop, and its characteristics are superimposed on their osteomalacia myopathy. CLINICAL CASES AND CONCLUSIONS: We report two patients with terminal chronic renal failure on hemodialysis who complained on difficulty with walking. The clinical findings, laboratory and neurophysiological investigations and the histopathological report of the muscle biopsy were similar to those of osteomalacia myopathy. The muscle weakness of patients with terminal chronic renal failure is related to many factors. The most important of these is the secondary hyperparathyroidism which leads to osteomalacia myopathy. On muscle biopsy there was isolated fibre necrosis of both types 1 and 2 (case 1) and of type 2 alone (case 2), which is considered to be the commonest finding. Although treatment with high doses of vitamin D3, or its hydroxylated metabolites, has been recommended, it was not effective in our patients.

Aged↗
PubMedDate not supplied

[Late symptomatic initiation of unilateral cerebellar hypoplasia].

A 65-year-old woman developed a progressive action tremor on her left hand for the last two years. On examination an intentional left hand tremor with dysmetria and tendency to deviate to left on tandem were identified. There was no mental deterioration. CT scan and MR imaging disclosed a marked unilateral, left, cerebellar hypoplasia. MR also showed multiple lacunae. Neurological family disease and exposition to drugs or alcohol were not present. In some cases cerebellar hypoplasia could become symptomatic at advanced age as result of concurrent cerebral disease affecting the compensating structures for congenital defect. In this patient a vascular mechanism is suggested as possible late trigger.

Aged↗
PubMedDate not supplied

[Cystinosis: an infrequent cause of distal myopathy].

INTRODUCTION: Cystinosis is a hereditary disease with clinical symptoms that are caused by the accumulation of cystine crystals in different tissues. Distal vacuolar myopathy has been reported as one of its later complications. CASE REPORT: Here, we present the case of a 20-year-old male diagnosed with cystinosis at the age of 2 years, with severe renal involvement that required a transplant. The patient gradually developed weakness and atrophy of the muscles in his hands. Neurophysiological and histological studies enabled a diagnosis of distal vacuolar myopathy to be established, and electron microscopy revealed deposits of cystine crystals. CONCLUSIONS: Cystinosis must be included within the differential diagnosis of distal myopathies. Timely treatment with cysteamine could prevent the development of this complication.

Adult↗