ABSTRACT
To study afferent and efferent mechanisms of both pathogenesis and forming of the symptoms of dystonia a clinical-electrophysiologic examination was performed in 22 aged 35-69 years patients with cranial dystonia. A wide range of different factors in anamnesis of the patients was found, that had had either short or long influence on the facial zone and that had preceded the development of cranial dystonia (2 month-5 years before its rise). This was called by the authors the peripheral factor of dystonia pathogenesis. This peripheral factor may have a starting and maintaining role in pathogenesis of dystonia. The results of electrophysiologic studies (electromyography, method of dermal sympathetic potentials, registration of abdominal reflexes) suggest the presence of diffuse hyperactivity of interneuronal apparatus on different levels of brain stem being, probably, an efferent link in both pathogenesis and symptoms of dystonia as a whole and cranial dystonia, in particular. The conclusion is made that a removal of the peripheral factor as a stable link of pathogenesis of cranial dystonia together with the influence on the efferent link of pathogenesis may be a new aspect in therapy of this disease.
Subject(s)
Dystonia/diagnosis , Dystonia/physiopathology , Muscle Tonus/physiology , Muscle, Skeletal/physiopathology , Adult , Age Factors , Aged , Anxiety/diagnosis , Blepharospasm/diagnosis , Blepharospasm/etiology , Blinking/physiology , Dystonia/complications , Electromyography/methods , Facial Nerve/physiopathology , Female , Head , Humans , Male , Middle Aged , Psychometrics , Severity of Illness IndexABSTRACT
Peripheral mechanisms of pathogenesis and formation of the symptoms of cervical dystonia (spastic torticollis--ST) were investigated. By means of orthopedic diagnosis and analysis of motor stereotype, traumas, algesic syndrome in 40 patients with ST, it was found a "short leg" on the side, opposite to the rotation of the head, frequent postural loads on the head and on the neck before ST rise, and algesic and other sensory symptoms in the same region. The data of neuromyography and of evoked skin sympathetic potentials show a presence of pathologic changes in sympathetic autonomic fibers as well as in axons of accessory nerve at both sides, mainly at the left. Such alterations were mainly peripheral and were found more frequently in a group of left-sided ST. The correlations observed between a side of the rotation of a head and a side of peripheral factor, permitted to suppose that peripheral factors (the factor of a "short leg" mainly) determined frequently a side of the rotation of the head. So, ST therapy must influence the peripheral mechanisms, but not only the central ones.
Subject(s)
Torticollis/etiology , Accessory Nerve/physiology , Accessory Nerve/physiopathology , Adolescent , Adrenergic Fibers/physiology , Adult , Axons/physiology , Electromyography , Evoked Potentials , Female , Functional Laterality , Humans , Male , Middle Aged , Posture , Rotation , Torticollis/physiopathology , Torticollis/therapyABSTRACT
A study was made of the clinical and electrophysiological data on 33 patients. In all the patients, motor disorders played the leading part. 18 patients manifested distal tetraparesis, 7 diffuse, 3 tetraplegia, and 5 had multiple mononeuropathy. The progressive, recurrent and monophasic types of the disease course were distinguished. The electrophysiological study revealed pathology primarily in muscles of the distal parts of the limbs and local areas of the blocking of excitation conduction in different nerves. The prognosis and treatment of the patients with glucocorticoids, cytostatics and plasmapheresis are under discussion. The catamnesis is 5 years.
Subject(s)
Demyelinating Diseases/diagnosis , Polyradiculoneuropathy/diagnosis , Adolescent , Adult , Aged , Child , Child, Preschool , Chronic Disease , Combined Modality Therapy/methods , Demyelinating Diseases/physiopathology , Demyelinating Diseases/therapy , Electrophysiology , Female , Humans , Male , Middle Aged , Peripheral Nerves/physiopathology , Polyradiculoneuropathy/physiopathology , Polyradiculoneuropathy/therapyABSTRACT
The authors describe a rare and little studied form (distal) of spinal amyotrophy. 12 patients were placed under observation. A group of patients with symmetric distal pareses of the hands and legs has been distinguished. Patients with long existent distal monoparesis of the hand or leg are described. The role of EMG in the differential diagnosis is emphasized.
Subject(s)
Foot/innervation , Hand/innervation , Hemiplegia/diagnosis , Muscles/innervation , Muscular Atrophy, Spinal/diagnosis , Adult , Diagnosis, Differential , Electromyography , Female , Hemiplegia/etiology , Humans , Male , Middle Aged , Muscular Atrophy, Spinal/complicationsABSTRACT
Clinico-electromyographic characterization of 20 patients with different forms of the spinal muscular atrophy (bulbospinal in 8 patients, chronic proximal in 5, distal in 7) is reported. The patients displayed the characteristic clinical signs and neuronal changes in EMG. The latter data are specified for each form. The authors infer that the spinal muscular atrophy in adults, children and adolescents fall into one group, though differing in the onset age, type of inheritance, clinical features, severity and prognosis.
Subject(s)
Muscular Atrophy, Spinal/diagnosis , Adult , Aged , Chronic Disease , Electromyography , Female , Humans , Male , Middle Aged , Muscular Atrophy, Spinal/classification , Muscular Atrophy, Spinal/genetics , Muscular Atrophy, Spinal/physiopathologyABSTRACT
Two clinical cases of post-poliomyelitic progressive amyotrophy (PPA) are described. One patient had serological tests of the blood and cerebrospinal fluid for viruses of poliomyelitis, measles, simple herpes and tick-borne encephalitis which were negative. The authors suggest that PPA develops in patients with a peculiar genetic predisposition and altered homeostasis and is a degenerative-dystrophic disease of the motor nerve.
