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PubMedDate not supplied

[Genetic investigations in facioscapulohumeral muscular dystrophy: a preliminary report].

Facioscapulohumeral muscular dystrophy (FSHD) is a primary muscle disorder with autosomal dominant inheritance. FSHD was mapped to chromosome 4 locus q35, but the gene is not yet known. It is characterised by progressive, often asymmetric, selective muscular weakness and great clinical variability. The aim of the study was to analyze 62 FSHD cases from 44 Polish families in which the diagnosis was confirmed by DNA analyses. FSHD diagnosis was based on the clinical findings and standardized investigations confirming primary muscular involvement (EMG, muscle biopsy). DNA analysis was based on EcoRI/BlnI restriction enzyme digestion followed by hybridization with P13E-11 molecular probe. In our material, we have found a relatively large percentage (41%) of big deletions (EcoRI/BlnI fragment of 10-15 kb [kilo bases]), which in the majority of cases (67%) was present in isolated cases. In 10 families (23%) the phenotype was assessed as severe. These are cases with the onset before the age of 10 and fast progression. "Middle sized" deletions (EcoRI/BlnI fragment of 16-29 kb) were prevalent in familial cases and present in 57% of families. "Small" deletion was found in one family (EcoRI/BlnI fragment of 30 kb). Somatic mosaicism was confirmed in one case. De novo mutations were shown in 11% of the examined families. The results of this study indicate that the bigger the deletion, the more severe the FSHD course, however there are some exceptions. A similar relationship has been shown by previous research. Molecular analyses are particularly important in atypical and sporadic cases. It is the first genetic presentation of a group of patients' with this kind of dystrophy in the Polish population.

Chromosome Deletion↗
PubMed1991

Muscle pathology in ankylosing spondylitis: clinical, enzymatic, electromyographic and histologic correlation.

We assessed muscle pathology in 30 patients with ankylosing spondylitis (AS) and 22 controls to assess if skeletal muscle is affected primarily by the inflammatory process of the disease. Investigations included a questionnaire on musculoskeletal discomfort, physical exercises, dynamometric measurements, EMG, and biopsy of the quadriceps muscle. Symptoms of muscular weakness were related with enthesopathic activity index. Plasma CK was higher in patients than in controls. A myopathic EMG pattern was found in 46.4% patients. Histological changes were found in 66% and did not correlate with symptomatology. Patients with AS with clinical muscular manifestations probably have intense enthesopathic inflammatory activity. It is suggested that muscles are secondarily affected as a consequence of pain inhibition and reduced activity.

Adolescent↗
PubMed1990

Clinical remission following plasmapheresis and corticosteroid treatment in a dog with acquired myasthenia gravis.

A 7-year-old sexually intact male Labrador Retriever with regurgitation and generalized muscular weakness resulting from acquired myasthenia gravis received 2 plasmapheresis treatments in combination with corticosteroid treatment. Plasmapheresis was performed in an attempt to rapidly lower serum acetylcholine receptor binding antibody (AChR Ab) concentration. Seven days after the second plasmapheresis treatment, the dog's muscular strength was normal, which coincided with a 70% decrease in serum AChR Ab concentration. Because the dog also received corticosteroids, it is impossible to determine how much of the clinical improvement resulted from plasmapheresis.

Animals↗
PubMed1996

Isokinetic strength following knee arthroscopy.

This study objectively evaluates recovery of quadriceps and hamstring strength following knee arthroscopy in a group of 43 patients given rehabilitation instructions, but limited supervised rehabilitation. Although the patients did not complain of weakness or functional limitation, isokinetic testing 1, 3, and 8 weeks following surgery revealed persistent weakness in many patients. Incomplete recovery was most apparent when patients were categorized by the degree to which they had recovered normal strength. Results expressed as a mean for the entire group indicated reasonably good recovery and obscured the significant weakness persisting in some patients. Knee extension strength tested at 60 degrees/sec had returned to normal 8 weeks after arthroscopy in only 32% of the subjects. Hamstring strength recovery was better, with 71% having normal strength. Patients may not recognize or report muscular weakness following arthroscopy, yet following 8 weeks of self-supervised rehabilitation many will have objective evidence of persistent weakness. In asymptomatic patients the clinical significance of this weakness is unknown.

Adult↗
PubMed1997

Myotonic dystrophy. The role of large triplet repeat length in the development of mental retardation.

OBJECTIVE: To describe mental retardation and microcephaly as initial clinical signs in myotonic dystrophy (MD) with high trinucleotide repeats. PATIENTS AND METHODS: Two patients with maternally inherited MD were examined. Southern blot analysis was performed and trinucleotide repeat expansions were related to the findings of clinical and magnetic resonance imaging investigations. RESULTS: Both patients had the large CTG trinucleotide repeat expansions often seen in congenital MD, but they lacked the typical clinical signs. Mental retardation and microcephaly were the leading features present in infancy. Muscular weakness, in contrast, developed after age 35 years. Although there was no evidence for perinatal asphyxia or sleep apnea, magnetic resonance imaging disclosed reduced brain volume and subcortical demyelination. CONCLUSIONS: Mental retardation preceding the development of muscle weakness suggests that the cerebral involvement in MD is a direct consequence of the genetic disorder and not mediated by muscle disease. Careful clinical examination of the parents for signs of MD should be considered in patients with cognitive deficits even without apparent muscular involvement.

Adult↗
PubMed1998

Myasthenic syndrome of snake envenomation: a clinical and neurophysiological study.

In this prospective study, 65 consecutive patients with neurological manifestations after snake envenomation, were examined in order to describe the natural history of the reversible nature of muscle weakness. Snake envenoming led to a completely reversible muscle paralysis involving the external ocular muscles with sparing of the pupils, muscles of mastication, facial muscles, palatal muscles, neck and proximal limb muscles. The deep tendon reflexes were preserved with no sensory abnormalities. The muscular weakness usually set in within an hour of envenomation and lasted up to 10 days, with fatigability lasting for 12 days. Respiratory muscle paralysis led to ventilatory failure needing ventilation in severely envenomed patients. Motor and sensory nerve conduction were normal with normal resting compound motor action potentials on electromyography. Repetitive nerve stimulation gave rise to a decremental response during high frequency stimulation. The edrophonium test gave negative results. These manifestations are due to abnormalities of neuromuscular transmission and are not typical of myasthenia gravis. As the exact pathophysiology of venom-related neurotoxicity is not known, it is suggested that the neurological manifestations of snake envenoming be designated a myasthenic syndrome. Further studies to isolate the neurotoxin and its mechanism and exact site of blocking at the neuromuscular junction would pave the way for the development of a novel long-acting neuromuscular blocking agent.

Action Potentials↗
PubMed1978

[Muscular involvement in osteomalacia: clinical, hystoenzymologic and ultrastructural study in 10 cases].

Osteomalacic myopathies are rare. They can prevail, however, and occur before bone abnormalities. The diagnosis must rest on clinical observation since the histopathologic images are not specific. On the other hand the demonstration of muscular weakness is very frequent during osteomalacia. In fact two types of manifestations correspond to the same anatomopathologic lesions. These are myopathic changes observed also during light microscopy, histoenzymologic and ultrastructural examination in the 10 patients examined. On the basis of these morphologic changes, muscular involvement can be considered to be part of the osteomalacia syndrome. The contribution of various factors including secondary hyperparathyroidism, vitamin D metabolism disorders, and phosphorus depletion is discussed. It is probable that many of them act together, causing reversible changes in muscular fibers. The intimate mechanisms of these changes are unknown.

Apyrase↗
PubMed1997

[The contribution of conventional EMG to the diagnosis of amyotrophic lateral sclerosis with bulbar symptoms].

INTRODUCTION: We presented here the results of quantitative electromyographic (EMG) study from 61 patients affected by Amyotrophic Lateral Sclerosis (ALS) with bulbar symptomatology at the time of study. MATERIAL AND METHODS: Patients were divided into two groups depending on whether the illness started with bulbar manifestations alone or with predominant neuromuscular weakness. RESULTS: Electromyography was performed on the abductor pollicis brevis, deltoideus or biceps brachii and tibialis anterior muscles (target muscles). In the first study the EMG was abnormal in more than 90% of the patients regardless to the duration of the illness and muscular weakness distribution. In the follow-up study the EMG was abnormal in all the patients lacking significant differences between the two groups. CONCLUSIONS: Quantitative EMG appeared as a very useful tool for the early diagnosis of ALS with bulbar manifestations. We found a correlation between the alterations of the different EMG parameters with the natural evolution of the illness.

Aged↗
PubMed1972

Neuromuscular involvement in pituitary gigantism.

Investigation of two pituitary giants complaining of severe muscular weakness showed a peripheral neuropathy in both cases. Histological appearances suggestive of myopathy were also present in one case.

Acromegaly↗
PubMedDate not supplied

[Thyrotoxic periodic paralysis. A report of 2 cases].

INTRODUCTION: Thyrotoxic hypokalemic periodic paralysis is characterized by recurrent episodes of motor weakness of variable intensity associated with thyroid overactivity. It is usually associated with low plasma potassium levels and is often precipitated by physical activity or ingestion of carbohydrates. CLINICAL CASES: We describe two men, aged 33 and 50, who complained of several episodes of muscular paralysis in the context of previously undiagnozed hyperthyroidism and associated with low plasma potassium levels. There were clearly raised levels of T3, T4 and free T4 and TSH was depressed due to hyperactive diffuse goitre. In one patient the precipitating factor was known to have been a large intake of carbohydrates and intense physical exercise. Antithyroid treatment, and the resulting correction of hyperthyroid function, prevented any further episodes of muscular weakness in both patients. CONCLUSIONS: Thyrotoxic periodic paralysis should be considered in the differential diagnosis of all acute episodes of motor paralysis in young patients. Determination of the plasma levels of potassium and thyroid hormones helps diagnosis. Early diagnosis is important so as to be able to establish antithyroid treatment and avoid further episodes of weakness.

Adult↗
PubMed2006

Hypokalemic rhabdomyolysis in a child due to amphotericin B therapy.

Hypokalemia can result in life-threatening complications if not treated appropriately. Although hypokalemia is a frequent adverse effect of amphotericin B therapy, there are no reports in the pediatric literature on hypokalemia-associated rhabdomyolysis induced by this drug. A ten-year-old boy with a history of one week amphotericin B treatment was admitted with weakness of the lower extremities, inability to walk and calf pain. Laboratory tests showed a serum potassium of 1.7 mEq/L and a serum creatinine phosphokinase of 3937 U/L plus myoglobulinuria. Following fluid expansion and intravenous potassium replacement, the patient progressed to achieve full regression of muscular weakness after one week. This report highlights hypokalemia as a rare cause of rhabdomyolysis. Patients on amphotericin B should be checked for this rare yet potentially life-threatening complication.

Amphotericin B↗
PubMed2004

[Muscular atrophy as a symptom].

Muscular atrophy is a symptom characterized by the loss of normal muscle mass. It is caused by a decline in the total number of muscle cells, or by a substantial decline in the substance of individual muscle cells. It has been associated with a preceding muscle weakness. Muscular atrophy occurs late in the course of a disease. Due to the limited ability of muscle cells to regenerate, it is frequently irreversible. Hence the aim is to detect the early stages of muscular decline and to prevent outright muscular atrophy. To achieve this it is necessary to be aware of the large number of diseases that can induce this in order to ensure timely referral to a specialist.

Diagnosis, Differential↗
PubMed1991

Autosomal dominant neuromuscular disease with cylindrical spirals.

Cylindrical spirals (CS) have been reported in muscle biopsies from five individual cases, as well as in two belonging to one family where there was another affected member, clinically associated with cramps, pain, stiffness and/or weakness. Here we studied muscle biopsies of a 70-yr-old mother and her 52-yr-old son, the latter with an associated neuropathy, both with late clinical onset in whose family at least 10 other members, spanning five generations, were diversely affected by muscular weakness, gait disorders, motor impairment and/or scoliosis, featuring an autosomal dominant trait with variable expression. CS as the main pathological findings were observed by light microscopy mostly in type 2 fibres, consisting of subsarcolemmal or intermyofibrillar granular and/or rod-like clusters, bluish with haematoxylin, bright red with Gomori's modified trichrome, non- or lightly reactive with PAS, faintly coloured with NADH-TR, non-reactive with SDH or ATPase, strongly stained with non-specific esterase and myoadenylate deaminase. Ultrastructurally, CS appeared as concentrically wrapped lamellae 1-2 microns in diameter. On occasion CS merged into tubular vesicular structures strongly resembling tubular aggregates (TA). Dilation of terminal cisternae (TC) in their proximity supports an origin from the sarcoplasmic reticulum (SR). Variable gene expression possibly explains both the highly diverse clinical compromise and time of onset.

Adult↗
PubMed1984

[Adult form of acid maltase deficiency presenting as progressive spinal muscular atrophy].

As far as could be elucidated, a sporadic manifestation of a slowly progressing muscular weakness and atrophy had commenced in a female patient symmetrically in the pelvic girdle and thigh region at the age of about 22 years. Only at the age of 55 years a precise neurological examination was done. On the basis of clinical and electromyographic data spinal muscular atrophy of the Kugelberg-Welander type was diagnosed. Muscular biopsy demonstrated severe far-progressed neurogenic muscular atrophy. In addition, increased storage of PAS-positive material was noticed. This was particularly true in the sparsely preserved, non-denervated muscular areas changed in the manner of an accompanying myopathy. Complementing electron microscopy and pathobiochemistry in the muscular biopsy showed an adult form of lack of acid maltase (Pompe's disease) aetiologically. This enzyme defect could also be demonstrated in the white cells of this patient.

Adult↗
PubMedDate not supplied

Primary biliary cirrhosis and myopathy: an uncommon association.

Primary biliary cirrhosis (PBC) is a cholestatic liver disease, which is characterized by a chronic inflammatory destruction of intrahepatic bile ducts. It is a rare disorder whose precise etiology is still to be elucidated. Even though the liver is the principal target of PBC, other organ systems also might be affected. Muscular involvement has rarely been described in this disease, and in the majority of cases, muscular weakness has been interpreted as polymyositis. We report the case of a 48-year-old woman suffering from classic PBC, in association with a myopathy whose histological features are distinct from the cases reported before. We also performed a MEDLINE research for PBC and concomitant muscular diseases.

Female↗
PubMed2000

[Steinert's myotonic dystrophy: severe neonatal form with unknown family history].

UNLABELLED: A child with severe generalized hypotonia and respiratory insufficiency, with an unknown positive family history for Steinert's disease, is referred. We want to point out the importance of correct anamnesis and of physical examination during pregnancy to suspect and diagnose rare and incurable fetal pathologies. CASE REPORT: The child was born after caesarean section. Polyhydramnios and decreased fetal movements were noticed during pregnancy. At birth, the baby presented asphyxia (Apgar 4/6) and respiratory insufficiency: he was then intubed. He received assisted ventilation for 37 days. At the physical examination, the child appeared hypotonic, hyporeflexic, without sucking reflex, with arthrogryposis and ligament laxity. On first day, chest X-ray showed paralysis of the right hemidiaphragm. His mother presented with hypotonia of the facial muscles, lid drop, light muscular weakness, positivity to neostigmine test: we then assumed that the baby was affected by transient neonatal myasthenia gravis and neostigmine was carried on. Anyway, the general conditions of the baby didn't improve. We were able to establish diagnosis of Steinert's disease (a form of muscular dystrophy with autosomal dominant inheritance with incomplete penetrance) after some other examinations (negativity of acetylcholine receptor antibodies, elevation of creatine kinase level, myopathic pattern on electromyography). Gene DMPK alteration was documented with a molecular genetic test.

Female↗
PubMed2002

[A case of dermatomyositis with significantly high level of KL-6 associated with ovarian cancer].

The patient was a 58 year-old woman. Starting about seven months before the initial examination, the patient began to experience a general malaise, coupled with reddish purple scaling eruption on the MP joints, knuckles, elbows, and knees, and dark purplish erythema on both upper eyelids. About two months before the initial examination, the patient visited a local medical doctor because she began to experience arthralgia and muscular weakness. Based on the condition of her weakening proximal muscles and increased levels of myogenic enzymes, the patient was diagnosed as having Dermatomyositis. Oral administration of PSL (25 mg a day) was initiated, and the patient was referred to our department to undergo thorough examination. Although deterioration in muscular strength and elevation of myogenic enzyme levels were mild during the initial examination, the level of KL-6 was significantly elevated to 2600 U/ml. Numerous blood gas analyses and chest CT did not reveal any exacerbation of interstitial pneumonia. Various tests were performed to determine whether or not there was any malignancy, and although a small amount of ascites fluid was detected, diagnostic imaging revealed no clear abnormalities. Since cytological diagnosis of the ascites fluid was class V, an exploratory laparotomy was performed, and ovarian cancer complicated by peritoneal metastasis was discovered. As a result, the patient was diagnosed as having dermatomyositis accompanied by ovarian cancer. The level of KL-6 in the ascites fluid was significantly high at 10,900 U/ml, and immunohistological staining using anti-KL-6 antibodies confirmed the presence of KL-6 in the ovarian tumor, thus suggesting that KL-6 was produced by the ovarian cancer.

Antigens↗
PubMed2003

[Postsurgical pain and weakness in the shoulder-arm region: iatrogenic or not?].

Four patients, one man aged 66 years and three women aged 69, 33 and 55 years, respectively, had postsurgical pain and weakness in the shoulder-arm region. Initially, a peripheral nerve lesion on a mechanical basis was suspected in all patients. However, because of the sharp pain starting after a postsurgical pain-free interval ranging from a few hours to two days, followed after some time by local muscular weakness, the diagnosis 'neuralgic amyotrophy' was made later. The electromyographic findings were in agreement with this diagnosis. The chronological sequence of the symptoms only became clear after targeted questioning. After 6-24 months, the strength was more or less restored. Usually, the prognosis of neuralgic amyotrophy is favourable, although full functional recovery may take two to three years and in a minority of cases recovery remains incomplete. Early diagnosis is important because of the prognostic aspects and to prevent unnecessary investigations or even surgical explorations, as well as legal claims. Therefore, not only neurologists but particularly surgeons and anaesthesiologists should be aware of this postsurgical condition.

Adult↗