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J G Lima

Publications and source records attributed to J G Lima.

At least 37 records · Page 2Linked to original sources
PubMed1992

Carrier detection of Duchenne and Becker muscular dystrophy using muscle dystrophin immunohistochemistry.

To ascertain whether dystrophin immunohistochemistry could improve DMD/BMD carrier detection, we analyzed 14 muscle biopsies from 13 DMD and one BMD probable and possible carriers. All women were also evaluated using conventional methods, including genetic analysis, clinical and neurological evaluation, serum CK levels, EMG, and muscle biopsy. In 6 cases, there was a mosaic of dystrophin-positive and dystrophin-deficient fibers that allowed to make the diagnosis of a carrier state. Comparing dystrophin immunohistochemistry to the traditional methods, it was noted that this method is less sensitive than serum CK measurements, but is more sensitive than EMG and muscle biopsy. The use of dystrophin immunohistochemistry in addition to CK, EMG and muscle biopsy. improved the accuracy of carrier detection. This method is also helpful to distinguish manifesting DMD carriers from patients with other neuromuscular diseases like limb-girdle muscular dystrophy and spinal muscular atrophy.

Adolescent↗
PubMed1991

[Congenital muscular dystrophy: histochemical study of the skeletal muscle in 17 patients].

A thorough histological description of 17 patients with congenital muscular dystrophy (CMD) is presented. The biopsies were performed in the left superficial deltoid muscle and processed with histochemical techniques. All samples showed connective tissue proliferation, changes in the internal architecture, necrosis, increase of adipose tissue, macrophagia, fiber regeneration and segmentation, central nuclei, and type I fiber predominance. The histological hallmarks of this entity are the marked endomysial connective tissue proliferation that frames one fiber from the other, and the important changes in the fiber's internal architecture. Those two abnormalities are extremely helpful to differentiate, on histological grounds, CMD from limb girdle muscular dystrophy and Duchenne/Becker muscular dystrophy. CMD presents a particular natural course and should be individualized apart from other muscular dystrophies.

Diagnosis, Differential↗
PubMed1991

[Congenital muscular dystrophy: clinical study of 17 patients].

We concur with the idea that congenital muscular dystrophy (CMD) is a distinct clinical entity, and report 17 patients (2 negroes and 15 whites; 12 M and 5 F; median age 6 years, range 1 to 24 years) with genetic, clinical, laboratorial, electrophysiological and histochemical studies. All our cases have an inheritance compatible with an autosomal recessive pattern. A decrease in fetal movements was reported by 57% of the mothers, generalized hypotonia at birth was present in 82%, limb girdle and neck weakness, absent or decreased deep tendon reflexes, and limb contractures were present in all. Severe muscular wasting was found in 41%. Calf pseudo-hypertrophy was observed in one patient. A patient was severely mentally retarded and another was borderline. During a 30-month follow-up, the muscle weakness of the majority remained essentially unchanged but the degree of motor activity deteriorated and was proportional to the worsening of the limb contractures. Serum CK levels were normal or increased to a maximum of 8 times. The electromyogram was myopathic in 74%, neurogenic in 13% and normal in 13%. CT scans showed a symmetrical white matter hypodensity in the hemispheres in 8 cases. All but 5 patients were operated upon to release the limb contractures and all were submitted to physical therapy. The contractures recurred in 4 patients submitted to surgery and were probably related to the cessation of physical therapy.

Adolescent↗
PubMed1991

[Mitochondrial myopathy: report of 12 cases with histochemical study of the skeletal muscle].

Twelve patients with histologically defined mitochondrial myopathy are described. There were 9 males and 3 females. The age of onset ranged from birth to 35 years with a median of 14 years. The most common clinical picture was that of ophthalmoplegia, ptosis and muscle weakness found in 10 patients. One presented with exercise intolerance due to muscular aches and pains, and the other besides his muscular weakness had mental retardation and an aggressive behavior. The clinical presentation and differential diagnosis of these patients are discussed.

Adolescent↗
PubMed1990

Muscle biopsy in AIDS and ARC: analysis of 50 patients.

We report a large spectrum of histological muscle abnormalities in 50 homosexual men with AIDS and ARC. We encountered moth-eaten fibers in 76%, angulated fibers in 76%, type groupings in 62%, type 2 fiber atrophy in 58%, mononuclear endomysial and/or perimysial and/or perivascular infiltrates in 36%, necrosis and phagocytosis in 30% and target fibers in 6%. Two patients showed vasculitis and two others showed perifascicular atrophy. On the whole, 96% of the biopsies showed substantial abnormalities. Muscle involvement in AIDS seems more common than previously suspected.

AIDS-Related Complex↗
PubMed1990

The action of prolyl-leucyl-glycinamide (PLG) on the nigrostriatal pathway of the rat.

In order to study the nigrostriatal pathway, we obtained the rotatory behavior model in male Wistar rats by electrolytic lesion of the left lateral hypothalamic region. Animals thus lesioned displayed rotations toward the same side of lesion when apomorphine was administered, a result in disagreement with what has been obtained in the model with 6-hydroxydopamine lesion. The administration of PLG alone was not followed by rotatory behavior but when the compound was administered in low doses (0.25 to 1mg/kg) simultaneously with apomorphine to animals previously submitted to REM sleep deprivation, a significant increase in the number of rotations was observed in comparison with controls and groups receiving higher doses of PLG. These results indicate that PLG may act as a modulator on dopamine receptors in the striatum.

Analysis of Variance↗
PubMed1988

Chronic exposure to the fungicide maneb may produce symptoms and signs of CNS manganese intoxication.

Manganese (Mn) poisoning, a well-known hazard in miners and industrial workers, shares many features with Parkinson's disease. Two young agricultural workers with a parkinsonian syndrome, who mentioned exposure to the fungicide maneb (manganese ethylene-bis-dithiocarbamate), led us to investigate a new possible source of Mn intoxication. Fifty male rural workers with occupational exposure to maneb were compared with 19 rural workers without fungicide exposure. We noted significantly higher prevalence of plastic rigidity with cogwheel phenomenon, headache, fatigue, nervousness, memory complaints, and sleepiness in the exposed group. In addition, we saw other neurologic signs, such as postural tremor, cerebellar signs, and bradykinesia, although without statistical significance. The data suggest that occupational exposure to pesticides containing Mn is a possible source of Mn intoxication of the CNS.

Adolescent↗
PubMed1988

Muscle biopsy correlated with electromyography. Study of 100 cases.

To find what the correlation is and verify if it is possible to avoid extensive electromyographic examination, studying only one muscle, 100 patients with neuromuscular disorders (58 primary myopathies, 32 neurogenic disorders and 10 myotonic dystrophies) were submitted to quantified electromyography (EMG) and muscle biopsy (MB) with fresh-frozen section plus histochemistry in the same muscle, but on the opposite side. The EMG was abnormal in 98% and MB in 93% of the cases. EMG and MB had a concordance of 84.3% in the neurogenic disorders and 84.77% in the primary myopathies. A correlation of 80% was obtained between all MB and EMG (including the cases of myotonic dystrophies), regarding the origin of the pathogenic process (p less than 0.01). The EMG had 5% inconsistencies and the MB 11%, with respect to the pathogenic process. When the myotonic dystrophy was separated from the primary myopathies and from the denervation disorders, a complete concordance was found in all MB and had only 3.4% inconsistencies in the denervation disorders and 3.1% in the primary myopathies.

Biopsy↗
PubMed1988

Correlation between specific histological and electromyographic findings in neuromuscular disorders.

An attempt was made to find a correlation between specific electromyography (EMG) abnormalities with histological findings in muscle biopsies (MB) in 100 patients with neuromuscular disorders. Quantified EMG and MB with histochemistry was made in the same muscle, but on the opposite side, within a period of 3 weeks. The isolated findings of EMG and MB were analysed with a computer through a chi-square test. A statistical relation (p less than 0.01) was found between the isolated findings of MB and EMG in only 6.99% (39 in 558 attempts) of the abnormalities expected to occur in myopathy and denervation. Also was found 2.51% (14 in 558 attempts) of inconsistences with the current literature.

Biopsy↗
PubMed1987

Rem sleep deprivation in an experimental model of Parkinson's disease.

Previous investigations have shown that REM sleep deprived (REM SD) rats display an enhanced response to dopamine agonists. This action seems to be mediated through a supersensitivity of dopamine post-synaptic receptors. Accordingly, REM SD was performed on rats with an experimental model of Parkinson's disease. The animals were bilaterally lesioned in the nigrostriatal pathway through a stereotaxically directed electrical current. Seven days after the surgery the animals were REM SD for 72 hours and immediately after the end of this period were observed in an open field for ambulation, rearing, grooming, and latency. In comparison with non-deprived rats there was a significant increase in ambulation and rearing, a response that appeared again after a second REM SD period on day 21th after the surgery. These data of improvement of two parameters of an experimental model of Parkinson's disease suggest that SD may be useful in this condition.

Animals↗
PubMed1987

Total sleep deprivation and Parkinson disease.

Twelve Parkinson disease (PD) patients were submitted to a single night of total sleep deprivation (SD). Disease duration had a median of 5.1 years and all were using either anticholinergic or L-Dopa or the combination of both drugs. After SD there was an improvement of rigidity, bradykinesia, gait and posture disturbances and functional disability that remained significant for 2 weeks. No effect was observed on tremor. Concerning depressive symptoms, a significant difference was noted, that remained for one week. These results suggest that SD may be an useful procedure to improve PD symptomatology. It is discussed a possible change of dopaminergic receptors, induced by SD, to explain the improvement.

Adult↗
PubMed1987

[Risk factors in headache in children from 7 to 15].

The occurrence of sleep troubles, recurrent abdominal pain, motion sickness, hyperactivity, dizziness, limb pain, cyclic vomiting, pseudoangine and the headache or migraine family history have been studied in 68 children migraine sufferers and compared to 68 non-headache sufferers whose ages range from 7 to 15. Data have revealed a significant predominance of those symptoms and family histories in migraine sufferers except pseudoangine which has had no significance, sleep troubles significant only in males and limb pains in females. The possibility of considering those factors as migraine risk factors is discussed.

Adolescent↗
PubMed1985

[Hypertensive intracerebral hematomas: apropos of 50 cases].

Fifty cases of supratentorial intracerebral hematomas associated with hypertension are analyzed. The best treatment of the intracerebral hematomas is not settled. The authors reviewed and analyzed the literature and they suggest that the small hematomas should be managed conservatively as well as those patients in deep coma. The subcortical hematomas usually improve with surgical removal. In the deep ganglionic type the initial treatment is clinical. If no improvement occurs the hematoma should be removed with microsurgery technique.

Adult↗
PubMed1985

[Intracerebral abscess: clinical treatment. Experience with a case].

A case of brain abscess treated non surgically is reported. Complete clinical and radiological resolution is demonstrated. The authors conclude that in selected patients conservative management of focal intracerebral infection may obviate the need for surgical intervention.

Brain Abscess↗
PubMed1981

[Carpal tunnel syndrome: electroneuromyographic study of 135 cases].

One hundred thirty five patients with carpal tunnel syndrome were studied trough electrophysiological techniques. Nerve sensory conductions, motor conductions and electromyography were performed in all patients and the results discussed. The authors call the attention for the technic of sensory conduction stimulating palma and picking up the potentials on the wrist as the most sensitive technic for the early detection of carpal tunnel syndrome.

Adult↗
PubMed1979

[Progressive extrinsic ophthalmoplegia; case report].

A case of progressive ophtalmoplegia and ptosis affecting a 12 year old boy is reported. A miopatic pattern was detected by the electromyography, the cerebrospinal fluid showed an increase of the protein content and retinal pigmentary degeneration was detected. This findings call the attention for a more widespread degenerative disorders affecting not only the muscular sistem but other organs as well.

Blepharoptosis↗
PubMed1978

[Vogt-Koyanagi-Harada syndrome: report of a case].

A case of Vogt-Koyanagi-Harada syndrome which had an ocular onset unilateral in the beginning and bilateral afterwards is reported. One month after the disease had appeared a psychiatric disturbance was detected characterized by mania and loss of the critical sense. The alterations detected in the eletroencephalograms and cerebrospinal fluid are discussed. After a three and a half years follow up, despite the corticotherapy the patient had a poor evolution on the ophtalmological aspect but the psychiatric recovery was total.

Adrenal Cortex Hormones↗