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Muscle weakness and paresthesia associated with epidural analgesia in a patient with an intrathecal neurofibrolipoma as part of a tethered cord syndrome.

We report a case of a 75-yr-old female patient in whom motor deficits and paresthesias occurred after lumbar epidural analgesia. These symptoms were eventually found to be due to a tethered cord syndrome. An epidural catheter was inserted for analgesia after colon surgery. The postoperative course was characterized by fluctuating sensory and motor symptoms. A magnetic resonance imaging scan showed an intraspinal mass, which was removed by laminectomy. The presented complication is of major interest because the intraspinal tumor, which must have been present for years, became acutely symptomatic. Tethered cord syndrome is caused by a limited longitudinal mobility of the cord. It is often seen as a part of spinal closure defects and is also associated with intrathecal tumors. Typically, adult patients complain of weak legs, paresthesias of the legs, and urinary incontinence. However, our patient had denied any muscular or neurological problems or urinary incontinence during the preoperative interview. Postoperative electromyogram and electroneurography ascertained chronic neurogenic lesions of multiple lumbar and sacral nerve roots. Three months after the operation, the patient was able to walk 100 m with a crutch.

Aged↗

Reappraisal of alcoholic myopathy. Clinical and biopsy study on chronic alcoholics without muscle weakness or wasting.

To determine if alcoholic neuropathy which causes denervation of the distal muscles of chronic alcoholics also produces a subclinical myopathy of their proximal muscles, we studied 11 chronic alcoholics who had no muscular weakness or wasting. Six patients demonstrated distal hyporeflexic (ankle jerks) sensory neuropathy on clinical examination. Four patients, one of whom was asymptomatic, had slow peroneal motor nerve conduction velocities. Patterns of neuropathy were present in the electromyograms of the proximal muscles of two patients. Muscle biopsy studies with enzyme histochemistry indicated denervation atrophy and myopathic changes in the contralateral quadriceps muscles of eight patients. As denervation atrophy was present, we concluded that these myopathic changes represented the effects of denervation of these muscles. We conclude, therefore, that the proximal subclinical alcoholic myopathy, previously described as primary by ourselves and others, is the result of denervation due to the well-known alcoholic neuropathy.

Adult↗

Skin rash and muscle weakness.

A 48-year-old Hispanic woman came to the clinic as a new patient--her chief complaint was a rash that appeared on her face 3 months before and had recently spread to her chest and hands. It itched occasionally and seemed to worsen after exposure to the sun. She also said that for the last month she had been feeling very weak-she had difficulty rising from a seated position and walking up the stairs to her apartment. She also felt as if her arms were heavy, making it difficult for her to brush and dry her hair in the morning. The patient was otherwise healthy with no known medical conditions, and she was not taking any medications. Her family history was noncontributory. What is your diagnosis? What diagnostic tests would you order for confirmation?

Dermatomyositis↗

[A case of NARP (neurogenic muscle weakness, ataxia, and retinitis pigmentosa) with a T-to-C point mutation at nt 8993 of mitochondrial DNA].

We report a case of NARP with a T-to-C point mutation at nt 8993 of mitochondrial DNA. A 37-year old man with mild mental retardation, retinitis pigmentosa, and clonic-tonic seizure was admitted to our hospital. The neurological examination revealed scanning speech, dystonic neck turning to the left side, and pyramidal tract signs. Serum-, CSF-lactate and pyruvate level were slightly elevated. Brain MRI findings showed cerebral atrophy, cerebellar cortical atrophy accompanied with dilation of forth ventricle, and high intensity lesions in the bilateral lenticular nuclei on T2 weighted images. Nucleotide sequence analysis of the mitochondrial DNA in the leukocytes demonstrated a T-to-C point mutation at nt 8993. To our knowledge, this is the first report of a Japanese patient with NARP associated with the T-to-C mutation at nt 8993 of mt DNA. Mitochondrial DNA analysis should be considered in the differential diagnosis of patients with retinitis pigmentosa and various neurological signs.

Adult↗

Periodic limb movement disorders and spells of profound muscle weakness due to airborne and dietary factors in humans.

Periodic limb movement disorders and the restless legs syndrome, generally considered to be sleep disorders, have a combined prevalence of almost 10% of the general population and are more common in women than in men. Although reduced dopamine activity in central nervous system motor control pathways seems to play a role, little, other than a list of associated risk factors, is known about the conditions that initiate the episodes. We report three patients, two teenage girls and one female teacher, who developed periodic limb movement disorders associated with high mold counts in a classroom and the gymnasium in the girls' school, and in the ventilating system in the teacher's school. Their disorders occurred when they attended their schools and cleared when they did not. These findings, combined with an earlier report of three cases where the disordered movements were triggered by dietary factors, suggest that in some patients, periodic limb movement disorders may be induced by inhalant as well as by dietary factors. Although these 6 patients may represent a subgroup of people with periodic limb movement disorders, potential dietary and environmental triggers should be considered in the clinical evaluation of patients seeking treatment for periodic limb movement disorders.

Adolescent↗

Effects of respiratory muscle weakness on daily living function, quality of life, activity levels, and exercise capacity in mild to moderate Parkinson's disease.

OBJECTIVE: To investigate respiratory muscle strength in mild to moderate Parkinson's disease in comparison with prediction equations and age-matched controls. To identify correlations between respiratory muscle strength and daily living function, activity levels, quality of life, and exercise capacity. DESIGN: A total of 66 participants with Parkinson's disease and 32 age-matched, healthy controls participated. Respiratory mouth pressures (representing respiratory muscle strength) were compared with predicted values. A comparison was also made between participants with Parkinson's disease and age-matched controls. Respiratory mouth pressures were correlated with results of the Barthel index (daily living function), a modified Baecke activity questionnaire (activity levels), the Parkinson's disease questionnaire (quality of life in Parkinson's disease), peak heart rate, peak oxygen consumption, blood lactate thresholds, and the number of stages completed during an incremental cycle ergometer test (representing exercise capacity). RESULTS: Respiratory mouth pressures were significantly lower (P < 0.05) in the Parkinson's disease group, but this did not influence the measures of daily living function, activity levels, and quality of life. Respiratory mouth pressures correlated with lactate thresholds (r = 0.308, P < 0.01) and the number of completed stages of the cycle ergometer test (r = 0.490, P < 0.01). CONCLUSIONS: In mild to moderate Parkinson's disease, there is a significant weakness of the respiratory muscles. This can affect the individual during exercise but has no apparent effect on activities that do not require a large effort of the respiratory muscles.

Activities of Daily Living↗

Natural history of muscle weakness in Friedreich's Ataxia and its relation to loss of ambulation.

From 1979 to 1992, 170 muscular assessments performed on 33 patients with Friedreich's Ataxia were reviewed. The average followup was 6 years. All muscle evaluations were done by the same team. It was possible to delineate a fairly regular and statistically significant pattern of slowly progressive and symmetrical loss of strength affecting mainly the lower limbs, and more specifically the pelvic girdle muscles. The first significant weakness was observed in the hip extensor group, followed in a variable fashion by other muscle groups of the lower limb. Upper limb and trunk muscles remained relatively spared until late in the disease process, with an overall strength approximately 80% of normal. Use of a wheelchair began at a mean age of 18.2 years, at which time the lower-limb strength averaged 70% of normal. Patients became totally unable to walk at a mean of age 20.5 years old, with a further decline in lower limb strength to 56% of normal. Weakness does not appear to be the primary cause for loss of ambulation in patients with Friedreich's ataxia.

Adolescent↗

Skeletal muscle weakness and dysphagia caused by acid maltase deficiency: nutritional consequences of coincident celiac sprue.

BACKGROUND: A 30-year-old woman with celiac sprue had progressive weight loss, myalgia, limb-girdle weakness, and dysphagia. METHODS AND RESULTS: Barium swallow showed an atonic esophagus, and scintigraphic study confirmed esophageal dysmotility. Skeletal muscle biopsy showed characteristic appearances of acid maltase deficiency, which was confirmed by a reduction of leukocyte acid alpha-glucosidase activity. CONCLUSIONS: Nutritional factors may have accelerated the presentation of the lysosomal storage disorder. This is the first reported case of dysphagia caused by esophageal motor weakness in acid maltase deficiency.

Adult↗

Muscle weakness after rest in myotonic disorders; an electrophysiological study.

Changes in amplitude of the evoked muscle action potential (MAP) have been observed in four patients with myotonia congenita and two with dystrophia myotonica. A fade in the response occurred in every case with stimulus frequencies of 10 per second or less, provided that the muscle was in a rested state and that long enough stimulus trains were used. Intramuscular stimulation and recording techniques show that the myotonic muscle fibre is the site of this defect. The MAP fade is thought to represent the transient weakness from which such patients may suffer, particularly after rest. Since neither this weakness nor the fade was related to the severity of the myotonia, nor were they significantly influenced by cooling or hydantoins, they may well be due to a separate defect in the myotonic muscle from that which causes the hyperexcitability of the fibre membrane.

Action Potentials↗

Usefulness of single fiber EMG for distinguishing neuromuscular from other causes of ocular muscle weakness.

Consecutive patients (n = 114), who had single fiber electromyography of the frontalis muscles for symptoms suggestive of ocular myasthenia gravis, were followed up for a mean of 14 months (3-64 mos). At follow up, based on strict criteria, 23 patients were classified as having ocular myasthenia gravis, 8 patients were diagnosed as having mitochondrial myopathy or oculopharyngeal dystrophy, 18 patients were found to have other diseases and 65 patients remained without a definite diagnosis. The single fiber electromyography data of these patients were then reviewed. The patients with ocular myasthenia gravis had, on average, more than 7/20 single fiber pairs with jitter > 45 microseconds and mean jitter of 56 microseconds. The 8 patients with mitochondrial myopathy or oculopharyngeal dystrophy had an average of 5/20 single fiber pairs with jitter > 45 microseconds and a mean jitter of 52 microseconds and could not be separated from the group with ocular myasthenia gravis on the basis of the single fiber electromyography results. The 18 patients with definite other diagnosis had an average of less than 1/20 single fiber pair with jitter > 45 microseconds and a mean jitter of 25 microseconds. This group could be clearly separated from the group with ocular myasthenia gravis. We conclude that single fiber electromyelography is useful in the separation of ocular myasthenia gravis from other causes of oculomotor weakness except mitochondrial myopathy and oculopharyngeal dystrophy.

Adult↗

Proximal muscle weakness in children.

In as many as half of the children referred to a muscle disease clinic, the original diagnosis is ultimately changed. Five cases histories are reviewed here in which Duchenne muscular dystrophy had been mistakenly diagnosed 3 to 12 years previously and the correct diagnosis was made only after repeat muscle biopsy or biopsy review. Four boys had diseases with a much better prognosis, and one boy did not have a muscle disease at all.

Biopsy↗

[Hang-back retroposition as a variation in oculomotor muscle weakness].

In a clinical study the authors evaluate efficiency of the operation on oculomotoric muscles by the hang-back retroposition method. A total of 70 children have been operated on, 52 of the patients suffering from esotropia and 18 from exotropia. The mean preoperation deviation in esotropia was 30 +/- 12 pdpt, the postoperation deviation was in the range of 6 +/- 7 pdpt. In the exotropia cases the preoperation deviation improved from original 76.9%, in the exotropia group being 77.8%. The overall success rate of the hang-back retroposition in all these patients was 77.1%. Ina group of children operated on by a traditional retroposition there was an overall success rate 78.7% (77.6% in esotropia and 82.4% in exotropia cases). Hang-back retroposition has been equally effective as the traditional retroposition of oculomotoric muscles. Its main advantages include visibility of the operation field, minimum risk of perforation of sclera and a fair time factor of the intervention. The authors discuss the development, indications, further advantages but also possible disadvantages or limitations of this acknowledged surgical procedure in strabology.

Child, Preschool↗

Respiratory muscle weakness in Charcot-Marie-Tooth disease. A field study.

A field study on respiratory muscle function and basic pulmonary mechanics was conducted at an international convention of 200 individuals with Charcot-Marie-Tooth disease. Forty patients with respiratory symptoms were evaluated; 15 had significant respiratory muscle dysfunction and 25 were normal. The results suggest that proximal upper limb involvement in individuals with Charcot-Marie-Tooth disease is a simple but significant predictor of respiratory muscle dysfunction.

Adolescent↗