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Results for “Muscular weakness”

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At least 73 records · Page 4Linked to original sources
PubMed1994

Late-onset muscular weakness in phosphofructokinase deficiency due to exon 5/intron 5 junction point mutation: a unique disorder or the natural course of this glycolytic disorder?

Late-onset muscle weakness is rare in glycolytic disorders. There are two reports in the literature of phosphofructokinase (PFK)-deficient Ashkenazi Jews with severe vacuolar myopathy manifesting in late adulthood. The genetic abnormality in these patients is unknown. We report a third patient with a similar syndrome: early-onset exercise intolerance in young childhood and progressive weakness in a limb-girdle distribution appearing at 57 years of age, leading to severe incapacity. Muscle histology showed diffuse vacuolar changes, and muscle fibers contained excess glycogen-like material. Muscle biochemistry was diagnostic for PFK deficiency. DNA analysis from the patient and his family showed that he was homozygous for a recently identified point mutation at the exon 5/intron 5 junction (a G-to-A change); two other family members were heterozygous for this mutation. It is not clear whether late-onset weakness is the natural course for all PFK-deficient patients or whether the exon 5 mutation carries increased risk for this severe myopathy.

Base Sequence↗
PubMed1993

Muscular weakness in the mdx mouse.

mdx mice are believed to be virtually free from neuromuscular symptoms, despite the presence of a degenerative/regenerative process that involves all skeletal muscles. We analyzed both the spontaneous motility and treadmill motor activity of mdx mice aged 15 days to 6 months. Our results indicate that there is an early period, between the end of the second and up to the fifth week of life, when mdx mice experience extreme weakness. After this critical period, both spontaneous motility and endurance of mdx mice, although lower than those of controls, do not show statistically significant differences up to 6 months of age. We also carried out a detailed histological analysis of proximal and distal muscle groups in mdx mice during this early critical motility period. The occurrence of extensive necrosis followed by regeneration and involving proximal muscles before distal ones was documented in mice as young as 16-17 days of age and reached a peak at day 18. We conclude that dystrophin deficiency induces muscle degeneration and significant weakness in mdx mice, but only in an early period. Later on, during development, mdx mice adapt to the lack of this protein and do not show detectable in vivo functional muscle impairment up to 6 months of age.

Aging↗
PubMed1993

[A case of flexion myelopathy presenting with reversible muscular weakness and atrophy of the unilateral proximal upper limb].

A 16-year-old man visited our clinic because of right-sided weakness of shoulder abduction and elbow flexion. He was well until about three weeks previously. Neurological examination revealed only that he had severe weakness together with mild atrophy of the muscles innervated by the C5 and C6 segments (the deltoid, biceps brachii, brachioradialis), slight hypesthesia on the thumb, and loss of deep reflexes of the biceps brachii on the right. No pyramidal signs were found in the lower extremities. EMG showed neurogenic changes of the atrophied muscles. Neuroradiological studies revealed right-sided atrophy of the spinal cord at C4-5 disk level, anterior shift of the posterior cervical dura mater and congestion of the internal vertebral venous plexus mainly at C4 and C5 vertebral level with his neck flexed. Conservative physiotherapy provided a good recovery within a few months. The clinical and neuroradiological findings of this case resemble those of juvenile type of distal and segmental muscular atrophy of upper extremities excluding distribution of involved muscles because of the different level of the spinal cord lesion. Pathomechanism of this case is considered to be the same as that of juvenile type of distal and segmental muscular atrophy of upper extremities.

Adolescent↗
PubMed1978

Transient muscular weakness in severe recessive myotonia congenita. Improvement of isometric muscle force by drugs relieving myotomic stiffness.

The maximum force of voluntary muscle contraction was registered under isometric conditions in nine patients with recessive myotonia congenita. The recordings were made on the upper arm. Five patients with severe myotonia had a transient weakness after muscle rest. Electromyographic registrations with wire electrodes showed that the myotonic muscle fiber discharges disappeared during the transient weakness. Medication improving myotonic stiffness also improved the weakness. The cause of transient weakness seems to be similar to that of myotonic stiffness. It is known that an increasing depolarization of the myotonic muscle fiber membrane leads to the myotonic discharges and myotonic stiffness. In severe myotonia the progressing depolarization could cause a loss of excitability of the muscle fiber membrane and thereby a transient paresis of a more or less large number of muscle fibers.

Adult↗
PubMed1989

The distribution of muscular weakness in upper motor neuron lesions affecting the arm.

The strength of 12 muscle groups of the arm was measured to determine the distribution of upper motor neuron weakness in man. Three groups of subjects were studied: 14 intact volunteers (both sides recorded), 10 patients with unilateral arm paresis (both sides recorded) and 6 patients with severe paralysis of the arm (the 'intact' arm only measured). On the side contralateral to the causative lesion the pattern of weakness was not the same in all patients. Shoulder muscles were relatively spared while the wrist and finger flexors were relatively severely affected, the difference being statistically significant. In hemiparetic and hemiplegic patients the strength of muscles ipsilateral to the lesion was reduced compared with normal controls. These observations refine previous clinical descriptions of upper motor neuron weakness and have implications for its pathophysiology.

Arm↗