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At least 1,531 records · Page 85Linked to original sources
PubMed1992

Role of electromyography in the diagnosis of motor neuron disorders.

Programming of electromyographic examination in motor neuron diseases is discussed taking into account application of appropriate techniques. The difficulties of correct interpretation of results are stressed. The stages of disintegration and reintegration of affected motor units are described as well as compensatory changes of surviving motor units. A detailed description of EMG dynamics of amyotrophic lateral sclerosis, late post-polio syndrome and of childhood spinal muscular atrophy is given.

Amyotrophic Lateral Sclerosis↗
PubMed2004

[The role of electrodiagnostic studies in the diagnosis of hypotonia in infancy].

Increased muscle extensibility and passivity characterize infantile hypotonia. It may reveal a peripheral neuromuscular disease as well as a disorder of the central nervous system. Electrodiagnostic studies in newborn and young infants are useful to guide the indication of other complementary investigations. Signs of denervation on needle electromyography strongly suggest infantile spinal muscular atrophy. Electrodiagnostic findings can distinguish rare conditions mimicking spinal muscular atrophy that are obstetrical tetraplegia and severe congenital neuropathies. Nerve conduction velocities are severely slowed in hereditary sensorimotor neuropathies and neurodegenerative disorders. Myopathic changes on needle electromyography are associated with congenital muscular dystrophies and structural or metabolic congenital myopathies. In congenital myotonic dystrophy, myotonic discharges can be recorded in the infant as well as in his/her mother. Myopathic changes may also be detected in collagen disorders, in cases of muscular atrophy secondary to hypomotility or malnutrition, and in patients with congenital myasthenic syndrome. Repetitive nerve stimulations are required to characterize myasthenic syndromes. Finally, normal results of electrodiagnostic studies constitute a relevant information that moves diagnostic procedures to search for central nervous system disorders. Benign congenital hypotonia is a quite rare condition that is diagnosed retrospectively, when hypotonia is strictly isolated and recovers completely before 2 years of age.

Diagnostic Techniques, Neurological↗
PubMed1990

ACTH and steroids in Kugelberg-Welander disease.

Two brothers with the chronic proximal form of spinal muscular atrophy (Kugelberg-Welander syndrome) were administered with ACTH and prednisone and a repeated remarkable and prompt improvement of the symptoms of this disease could be registered. Since similar experiences has recently been reported by other authors in hereditary sensory and motor neuropathy, a hypothesis is put forward of the possible participation of autoimmune mechanisms in the pathogenesis of some forms of hereditary so-called degenerative diseases of the peripheral motor neuron.

Adrenocorticotropic Hormone↗
PubMed1990

[MRI findings of the tongue in neurodegenerative diseases with bulbar sign].

We examined the magnetic resonance image (MRI) of the tongue in cases of amyotrophic lateral sclerosis (ALS), bulbo-spinal muscular atrophy (SBMA) and Shy-Drager syndrome (SDS) with sleep apnea. ALS case with severe bulbar disorder showed atrophic, irregularly margin tongue with increased signal intensity in T1-weighted MRI. ALS with less severe bulbar disorder demonstrated mixed hyperintensity and normointensity areas in the tongue. ALS without lingual symptoms showed increased intensity of tongue which suggested subclinical involvement of lingual muscle. BSMA case also showed atrophic tongue with diffusely increased signal intensity. SDS case with sleep apnea was revealed to have round shaped tongue without increased signal intensity, but his tongue fell into the posterior oral cavity, indicating hypotonus of genioglossus muscle. We concluded that MRI of the tongue is useful to determine the bulbar symptoms due to lower neuron disorder or not.

Aged↗
PubMedDate not supplied

[Pregnancy and labor in chronic anterior horn lesion].

This is the case of a 35-year old woman who suffers from a chronic form of spinal muscular atrophy and who has successfully completed four pregnancies between 1982 and 1987. After a short discussion of etiology, pathology, heredity and the differential diagnosis we describe the obstetric problems of our patient. The pregnancies were mainly aggravated by recurrent cystitis. In all the four deliveries the patient had to undergo cesarean section followed by severe lung problems. After the third pregnancy the patient had a nephrectomy cause of a pyonephrosis. We could not see an aggravation of the disease at it is described in some cases of Charcot-Marie-Tooth disease or polyneuritis. Three of the four children showed a striking muscular hypotonia post partum that disappeared soon after the neonatal period.

Adult↗
PubMed1988

Association of lower motor neuron disorders with fasciculation, neuromyotonia and myoclonus.

Fasciculations may be generated at any point on a hyperexcitable lower motor neuron. Physiological ('benign') fasciculations often begin suddenly and persist for years without development of muscular wasting or weakness. Fasciculations may be a sign of degeneration of lower motor neurons in which case they may be associated with muscle cramps and neuromyotonia. Both sensory and motor axons are overactive in neuromyotonia, the symptoms of which are relieved by administration of anticonvulsants such as carbamazepine. Spinal muscular atrophy is a feature in some cases of progressive myoclonic epilepsy of the systems degeneration type that may be overlooked because muscular wasting is overshadowed by the dramatic appearance of action myoclonus.

Brain↗
PubMed1987

[Familial distal progressive spinal amyotrophy with asymmetry of the lower limbs].

Two cases of spinal muscular atrophy, localized on the lower limbs and strictly unilateral, in two half brothers, are reported. Such a distribution of clinical changes and such a hereditary transmission appear to be unusual in hereditary motor-neuronopathies. A recessive heterotopic transmission may be suspected. To our knowledge, it has been reported in Kennedy's disease only.

Adolescent↗