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Biomedical subjects

D J Calzada-Sierra

Publications and source records attributed to D J Calzada-Sierra.

At least 19 recordsLinked to original sources
PubMedDate not supplied

[Stroke in a child due to hemoglobinopathy AS].

INTRODUCTION: Falciform cell anaemia is a genetically determined haemoglobinopathy which in homozygote form (HbSS) is accompanied by neurological disorders in a quarter of the patients, mainly in the form of cerebral ischemia. Some authors consider the heterozygote (HbAS) form to be asymptomatic, although several such patients have been described with stroke. CLINICAL CASE: A white boy of 5 years of age had a cerebral infarct whilst travelling by car from his home town (222 meters above sea level) to the capital of the country (which is 2,600 meters above sea level). During the journey he complained of headache, vomiting and the onset of right hemiplegia. Angio-MR and CT showed the characteristic features of an infarct. SPECT showed hypoperfusion of left frontal predominance and of the basal ganglia bilaterally, but with left-sided predominance. Electrophoresis studies of his haemoglobin showed the present of AS haemoglobinopathy. We present the results of investigations done and a review of the literature. CONCLUSIONS: AS haemoglobinopathy, together with hypoxia due to altitude and possibly slight dehydration, were probably the causes of our patient's cerebral infarct. We recommended electrophoresis of haemoglobin in all patients (especially children and young adults) who present with stroke.

Anemia, Sickle Cell↗
PubMedDate not supplied

[Neurological Behcet syndrome. Presentation of three cases].

INTRODUCTION: Behçet's disease is a multisystemic illness in which neurological abnormalities are seen in 5-48% of cases. CLINICAL CASES: We present 3 patients (2 men and 1 women) diagnosed as having Behçet's disease, according to the criteria of the 'International Study Group for Behçet's Disease' of 1990. We describe the clinical findings in these patients, the results of investigations done (MR or CT, evoked potentials-PEV, PESS, PEATC-EEG, ECN, CSF and neuropsychological tests), their course and response to treatment with hyperimmune human gammaglobulim at a dose of 400 mg/kg body weight in 2 patients. All three patients had repeated episodes of meningitis and alterations of the cranial nerves; two patients had epileptic seizures and two had signs of cerebellar disorders and dementia at some time during their illness. One patient died and the other two survived with severe disabilities. Cranial CT showed a cerebral infarct in one patient; evoked potentials showed axon damage in the two patients in whom this was studied. In the only patient in whom ECN was done, signs of axonal polyneuropathy were found. In all three patients there was pleocytosis in the CSF. In the patients in whom hyperimmune human gammaglobulin was given, the results were: one improved after this treatment; in the other there was clinical deterioration, but this was considered to be secondary to a curettage done the day before. CONCLUSION: The presence of neurological findings in Behçet's disease not caused by cerebral vein thrombosis worsens the prognosis of these patients.

Adult↗
PubMedDate not supplied

[Bilateral cyclic sciatica caused by endometriosis. Apropos of a case].

INTRODUCTION: Cyclical sciatica due to implantation of endometrial tissue in the sciatic nerve in the region of the sciatic notch is a very unusual cause of sciatica. It occurs in women of childbearing age, as episodes of pain in the distribution of the sciatic nerve, which present in a cyclic manner and coincide with menstruation. If it is not treated, a sensomotor mononeuropathy of the sciatic nerve develops. CLINICAL CASE: The patient had complained of right-sided sciatic pain from the age of 36 years. Over the years a motor deficit had slowly and progressively appeared causing foot drop. The painful crises were related to her menstrual periods. At the age of 44 years a pyramidal muscle syndrome was diagnosed and treated surgically. This was followed by increase in the crises of sciatic pain. A year later, she started to have sciatic pain on the left side, which was similar to that of the right side. The clinical, imaging and electrophysiological findings are reported. The patient improved. She is still being treated with depot medroxyprogesterone and her pain has disappeared. CONCLUSIONS: Cyclical sciatica due to endometriosis is little known and may lead to permanent disability. Computerized axial tomography of the pelvis using contrast material is very useful for diagnosis. The use of depot medroxyprogesterone seems to be a satisfactory treatment in some patients.

Endometriosis↗
PubMedDate not supplied

[Juvenile amyotrophic lateral sclerosis. Apropos of a case].

INTRODUCTION: There are scanty reports on juvenile forms of amyotrophic lateral sclerosis, specially amyotrophic lateral sclerosis and deafness, and it is known as Madras pattern of motor neurone disease. CLINICAL CASE: We describe an sporadic case of juvenile amyotrophic lateral sclerosis with deafness in a young person who started with hearing loss at 21 years old, loss of strength in upper limbs and muscular atrophy. He was seen by a neurologist when he was 25 years old, there were evident generalized fasciculation activity in proximal and distal muscles in the four limbs and the tongue, with swallowing troubles, and increased tendon reflexes in lower limbs with abnormal plantar extensor responses. All the paraclinical test were normal, except the electromyogram, showing a classical pattern of lower motor neuron disease, and the auditory brain stem response with absence of the main components of this evoked response, as expression of VIII cranial nerve damage. DISCUSSION: Patients like this one were first described in Madras (India), and the evolution of this kind of juvenile form of amyotrophic lateral sclerosis is chronically progressive and relative benign, in relation to the classical form of amyotrophic lateral sclerosis and other forms of motor neurone disease which begin in childhood, adolescence or young adulthood. CONCLUSION: Its recognition is very important in order to diminish misleading therapies in these patients.

Adult↗
PubMedDate not supplied

[Tarsal tunnel syndorme. A report of 3 cases].

INTRODUCTION: The tarsal tunnel syndrome is uncommon. It is a neuropathy of the tibial nerve at the level of the tarsal tunnel. The main symptom is pain of the sole of the foot. On percussion Tinel's sign may be found, with sensory loss all over the sole of the foot, or in parts of it, together with weakness of the intrinsic muscles of the foot. In all three cases nerve conduction studies were done and needle electromyography was also done in two cases, confirming the clinical diagnosis. CLINICAL CASES: The three patients were treated conservatively (rest, vitamin B complex and antiinflammatory analgesics). Two patients improved but one required surgical treatment when medical treatment alone was found not to be satisfactory. At the present time, all three patients are asymptomatic. CONCLUSIONS: The tarsal tunnel syndrome is uncommon but not unimportant. In most patients it presents as a syndrome of intense pain of the sole of the foot, with typical clinical features, usually unilateral. Nerve conduction studies and needle electromyography are particularly useful for confirmation of the diagnosis by localization of the problem. Initially conservative treatment is indicated in all cases. Those which do not respond to this will require surgical intervention.

Adult↗
PubMedDate not supplied

[Guillain Barre syndrome].

OBJECTIVE: To review about this disorder, with emphasis on the intensive care of severe Guillain Barr syndrome (GBS). DEVELOPMENT: GBS is an acute immune mediated inflammatory polyneuropathy that may lead to quadriparesis, ventilatory failure, and autonomic dysfunction but also to many general medical problems that have great bearing on outcome. Therefore severe GBS patients require admission into an intensive care unit (ICU), where in addition to the disorders mentioned before, other complications can arise. The neurologist who plans to deal comprehensively with these patients must be familiar with therapy for infections, nutrition, fluid management, and selected aspects of pulmonary medicine as well as the indications for and complications of plasma exchange and gammaglobulin infusion. CONCLUSIONS: With modern intensive care support, the outcome is excellent (>80% recovery), although in many cases a persistent residual paresis occurs. Because GBS is largely self limited, the skill daily cares of these patients in an ICU contributes as much, or more, to the overall outcome of an individual patient as do specific immune therapies.

Critical Care↗
PubMedDate not supplied

[Corticospinal dysfunction in patients with primary and amyotrophic lateral sclerosis].

INTRODUCTION: Corticoespinal dysfunction is a common finding in primary and amyotrophic lateral sclerosis (PLS and ALS). AIM. To compare the behaviour of motor evoked potentials (MEP) with transcranial magnetic stimulation (TMS) in patients with ALS and PLS. PATIENTS AND METHODS: It was performed a retrospective analysis of MEP recordings of 11 patients with PLS and 10 patients with sporadic ALS. Central motor conduction time and amplitude ratio were the selected variables for the statistical analysis of MEP from abductor pollicis brevis and tibialis anterior muscle from the four limbs, using non-parametric methods. RESULTS: As a general observation there was a high incidence of abnormal recordings in both groups of patients; in 30% of recording from ALS patients response to TMS was absent, but only the 4.5% in the group of PLS had the same characteristic. In PLS patients abnormal central motor conduction time was the most frequent finding, as it was the low amplitude ratio in ALS patients; both variables showed statistically significant differences between groups (Kruskall-Wallis, H = 6.32, p = 0.011; and Kruskall-Wallis, H = 5.777, p = 0.0163, respectively). CONCLUSION: Corticoespinal dysfunction has different characteristics in ALS and PLS patients, and the analysis of MEP could add useful information for differential diagnosis of these diseases.

Aged↗