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Biomedical subjects

R Alberca

Publications and source records attributed to R Alberca.

At least 19 recordsLinked to original sources

Episodic cluster headache and narcolepsy: a case report.

A patient who first presented with episodic cluster headache later developed narcolepsy. In spite of REM sleep alterations associated with narcolepsy, the frequency and distribution of pain attacks did not change when narcolepsy occurred and were similar to those seen in cases of episodic cluster without narcolepsy. The lack of influence of narcolepsy on the pattern of cluster pains questions the role of REM sleep states in triggering pain in episodic cluster.

Adolescent

Continuous muscle activity and distal spinal muscular atrophy.

A young man presented with myokymias, cramp-like difficulty in muscle relaxation and peroneal atrophy. EMG studies revealed continuous muscle activity (CMA) manifested as grouped potentials and high frequency discharges. Sensory nerve conduction studies and sural nerve biopsy gave normal results, and he was thought to suffer from distal spinal muscular atrophy with CMA. This association suggests that the lower motor neuron may have an important role in the generation of the continuous muscle activity.

Adult

[Neuralgiform paroxysmal migraine].

A 23-year-old woman presented with episodic, strictly unilateral, left-sided headaches of excruciating severity. Pain was referred to the eye, lasted from seconds to a minute and was accompanied by homolateral ptosis, redness of the eye, increased lacrimation and nasal discharge. The attacks of pain repeated up to seven times over 24 hours and clustered around ovulation for seven days a month. After she became pregnant, the attacks increased in frequency and appeared every five to ten minutes during night and day. Different medical treatments including indomethacin, were without effect. Two months later pregnancy was interrupted and the pain immediately subsided. After carotid angiography the pain reappeared for two months, but it finally disappeared and she has been free of pain without treatment for the last nine months. This syndrome can be related to episodic paroxysmal hemicrania.

Abortion, Induced

[Multifocal demyelinating neuropathy with persistent conduction blocks and continuous muscular activity].

A patient developed an initially asymmetric sensory-motor neuropathy, with definite predominance in upper limbs. The examination also disclosed a markedly impaired muscle relaxation. The neurophysiological study showed conduction blocks with continuous muscle activity consisting of myokymias fasciculations and muscle cramps which disappeared after the anesthetic block of the distal nerve segments. In the sural nerve biopsy significant abnormalities were not found. After prednisone and carbamazepine therapy in usual doses the symptoms reverted and the conduction blocks persisted. Neuropathy with persistent conduction blocks is an uncommon disease which exceptionally results in a clinically apparent syndrome of continuous muscle activity. Its recognition is important, as symptoms can disappear after correct therapy.

Adult

[Pregnancy and benign intracranial hypertension].

Several papers have suggested that pregnancy is one of the etiopathogenic factors of benign intracranial hypertension (BIH). The therapeutic attitude to be taken as regards new pregnancies in women previously afflicted with BIH during pregnancy is still on discussion. This paper is based on a study of 100 BIH cases. The results support the idea that cases, but the obesity involved. The coexistence of BIH and pregnancy does not increase the risk of relapse, does not mean a worse prognosis of BIH nor does it appear to have a negative effect on the child. Any woman who has previously developed BIH during pregnancy should not be advised against future pregnancies. In the case of a new pregnancy, a very close control should be carried out in order to avoid an excessive increase in weight.

Adult

[Recurrence of benign intracranial hypertension].

The recurrence of benign intracranial hypertension (BIH) in 100 patients was analysed after a long-term follow-up. A recurrence appeared in 20% of the cases in this series. This being more frequent, with statistical significance, in females aged between 20-40 years. Obesity was the more frequent etiopathogenic factor involved in the onset of relapses. Other factors were pregnancy, steroid therapy, levothyroxine and obstruction of the cerebrospinal fluid derivation system. Relapses did not produce loss of sight. We insist on the elimination of developing factors in order to prevent the recurrence.

Adolescent

[Benign intracranial hypertension and obesity].

The incidence of obesity and excess weight were analyzed in a series of 100 cases of benign intracranial hypertension (BIH) and compared with a control group. The results of hypocaloric diet and lumbar puncture were compared to those obtained with other treatments. Relapses of the disease were analyzed in obese patients who maintained a normal weight after clinical recovery and compared with those who get fat again. A similar analysis was carried out in pregnant women. The frequency of sequelae in obese patients was also determined. A high incidence of obesity was observed only in women aged 20 to 40 years who presented BIH. Hypocaloric diet and lumbar puncture constituted the treatment which exerted a more marked shortening of the clinical course. Relapses of BIH were more frequent in obese patients who did not normalize the body weight and in new pregnant women. Data indicate the relevance of obesity as etiopathogenetic factor in a group of patients with BIH.

Adolescent

[The state of muscle cramp disease].

A 65-year-old man presented with daily, almost continuous muscle cramps and painless muscle contractions eight years after being diagnosed as having rheumatoid arthritis. Both cramps and contractions were present at rest, were accentuated by stress and disappeared during sleep. By night-fall the patient was plunged into an extremely disabling condition due to the continuous cramps present in the orofacial, trunk, neck and limb musculature. He even had difficulty speaking. EMG studies demonstrated that both cramps and painless contractions appeared synchronously in muscles innervated by different peripheral nerves. A state of central hyperexcitability is the probable cause of this clinical picture which has remained unchanged over the last six years.

Aged

Late onset parkinsonian syndrome in Hallervorden-Spatz disease.

Two siblings, from consanguineous parents, developed in their twenties a Parkinsonian syndrome. In the elder, the disease evolved for 13 years and the necropsic study was diagnostic of Hallervorden-Spatz disease. The younger sibling is severely affected after 12 years of the disorder. Several CT and one MR studies done in this patient during the last 4 years have been normal. Ultrastructural studies of the bone marrow histiocytes and blood lymphocytes disclosed peculiar inclusions. Bromocriptine in low doses proved to be a beneficial therapy for this patient.

Adult

Brachial diplegia in central pontine myelinolysis.

A patient developed weakness in the upper limbs, eventually causing brachial diplegia with only slight paresis of the legs after rapid correction of severe hyponatraemia. Pseudobulbar palsy, mental confusion and urinary incontinence were also present. CT scan showed a zone of lucency in the pons. Clinical recovery occurred and the zone of lucency had disappeared 12 months after the appearance of the neurological signs.

Aged

Hereditary distal myopathy with onset in early infancy. Observation of a family.

The study of a family affected with hereditary distal myopathy with onset in early infancy is presented. Complete neurological examination was necessary in several members of the two last generations to discover the existence of the abnormalities of which they were unaware. The propositus was the most affected member of the family iwth distal paresis of the upper and lower extremities and selective paresis of the deltoid muscles. In addition he had kyphoscoliosis, talipes valgus and limitation of mobility of several joints. The onset of the disease was estimated as before the age of 2 when the child started walking. There was no progression of the disease. Clinical examination suggested a myopathic origin of the condition. A sural nerve biopsy was normal. Light-microscopy histochemical studies disclosed a predominance of type I fibres which were at the same time hypotrophic. Subsarcolemmal deposits of mitochondria were present although they were scanty and of normal ultrastructural appearance. In view of the morphological presentation it is postulated that this disease should be classified within the groups of myopathies accompanied by disproportion of fibres and selective atrophy of type I fibres.

Adult

Myotonia in centronuclear myopathy.

Centronuclear myopathy, which is unusual because of clinical myotonia, is described in two sisters. The diagnosis was established in adult life, but the first symptoms were noticed in infancy. The outstanding points of the clinical picture were mild amyotrophy, paresis, and clinical myotonia.

Adenosine Triphosphatases