[Demyelination of nerve fibers in pathogenesis of peripheral nervous system diseases].
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Sarcoid is a granulomatous disease of undetermined etiology characterized by the presence of epithelioid cell aggregates without caseation which proceeds to conversion to hyaline fibrous tissue or resolution. The sites of nervous system involvement include the meninges, cranial, and peripheral nerves, hypothalamus and pituitary gland, muscles, and, more rarely, brain and spinal cord parenchyma. In nervous system involvement there is usually cerebrospinal fluid lymphocytic pleocytosis and elevated protein but these findings are not specific. When other systems are involved with sarcoid the diagnosis of neurosarcoidosis is obvious. When an unusual neurological symptom complex presents, sarcoid should be considered. The author reviews the literature and presents 22 cases of sarcoid involvement of the nervous system.
Autonomic and pudendal neuropathies are frequent and neglected diseases affecting the genitourinary system. In the initial stages they frequently are asymptomatic. Autonomic neuropathy is seen in association with peripheral neuropathy in a wide range of metabolic diseases, including diabetes mellitus. Diagnosis can only be made directly by electrophysiologic methods. Treatment is based upon an adequate laboratory study of genitourinary function.
Three cases of non-healing granuloma with neurological complications are described. One case suffered from Stewart's form of the disease and two from Wegener's variety. The literature is extensively reviewed and the incidence and manner of neurological involvement in 374 cases is discussed. We suggest a classification to indicate four forms of nervous system involvement. First, granulomatous lesions of the central nervous system. Second, vasculitis of the central nervous system. Third, vasculitis of the peripheral nervous system and fourth, infection of the central nervous system. Of all cases 21 per cent had some form of neurological complication. Wegener's type showed more frequent neurological involvement that Stewart's, 26-5 and 12 per cent respectively. Infection of the central nervous system was limited to cases of the Stewart variety.
Intermittent claudication of neurogenic origin can be traced to three basic causes: --either a chronic circulatory deficiency in the arteries leading to the spinal cord, whether these arteries be the site of an atheroma of the ostia, an inflammation or a loco-regional compression. In such cases, the claudication is painless which differentiates it from the arteritis claudication of the lower limbs. --or to a compression of the roots of the cauda equina and to a lesser degree of the spinal cord, through a narrow rachidian canal that is hereditary or acquired, and relative or absolute. --or finally to a sheathing peripheral neuropathy of the lower limbs. The two latter causes are accompanied by pain, and make it necessary to widen the classical notion of the intermittent claudication (Dejerine's non painful intermittent claudication of the spinal cord).
A quantitative examination of sensitivity with using modulated sinosoidal currents was carried out in 10 healthy individuals and 44 patients with various disorders of the central and peripheral nervous system. In the healthy probands the magnitudes of the current force causing the specific sensation under the electrodes in various body regions were determined. A substantial, statistically significant rise of the current perception threshold was revealed in patients with superficial sensitivity disturbances. In patients with deep sensitivity disturbances both quantitative and qualitative distinctions were discovered.
A new SER recording system using the bilateral simultaneous peripheral nerve stimulation on cervical, thoracic and lumbar segments was found to be helpful as a diagnostic aid for the localization and the level diagnosis of sensory neuronal lesions including peripheral nerves. SER abnormalities were observed in 32 (76.2%) of the 42 cases with cerebrovascular disease, in 11 (64.7%) of the 17 cases with spinal cord lesions and 12 (75.0%) of the 16 cases with peripheral nerve lesions. As an interesting finding, marked SER abnormalities corresponding with the level and the distribution of the lesions were observed in cases with spinal cord tumor, myelopathy and polyneuropathy. In a case with recurrent myelitis, lesions of different levels of spinal cord were suggested from the SER changes. The recording system gives also valuable information about chronological modifications of the lesions, and may reveal subclinical neuronal damage in a certain neurological disorder.
Peripheral nerve tumors comprise less than 5% of all tumors of the hand. The most common solitary nerve tumor is the neurilemmoma, which arises from the neural sheath, is well encapsulated, minimally symptomatic, and may be surgically enucleated without producing a neurological deficit. Neurofibromas may be solitary, multiple, or associated with von Recklinghausen's disease. They are usually centrally placed with nerve fibers traversing the tumor mass making it more difficult to remove the tumor without producing permanent neurological damage. Malignant tumors include neurofibrosarcomas which often are very aggressive, requiring wide excision or amputation, and the rare neuroepitheliomas. Reported nerve tumors, intraneural in location but nonneural in origin, include fibrofatty infiltration of the median and digital nerves, intraneural lipoma, hemangioma, and ganglion cysts. These lesions may be treated by decompression or excision, depending on the nature of the tumor. Four unusual cases are described.
We studied five patients (two men and three women, age between 58 and 76 years) with clinical and electrophysiological signs of polyneuropathy. Routine neurological, hematological, and gastroenterological studies as well as procedures to test fat malabsorption were performed. Folate determinations were done using both radioactive and Lactobacillus casei methods. Two patients displayed the signs of subacute combined degeneration of the spinal cord with polyneuropathy, while three had only signs of neuropathy. All had low serum folate concentration, long-standing gastrointestinal disease, and deficient folate intake. The D-xylose absorption test gave values in all patients, while none displayed the classical malabsorption syndrome. The patients had substantial improvement or recovered (according to clinical and electrophysiological measurements) after periods ranging from 9 to 39 months of folate therapy. Such acquired folate-responsive polyneuropathy has two principal characteristics: mixed sensorimotor with mainly sensory deficits, and involvement of one or both of the lower extremities much more extensively than the upper extremities.
Three-month-old WAG/Rij rats were irradiated with 300 kV X-rays on the lumbar region of the spinal column with doses below the level for causing paralysis due to radiation radiculomyelopathy. 8--9 months after irradiation, degeneration of predominantly the ventral nerve roots of the cauda equina was boserved. Three stages were distinguishable: I) Demyelination and proliferation of Schwann cells; II) Local swelling of ventral nerve roots, with concentric layers of Schwann cells resembling hypertrophic neuropathy; III) Malignant Schwannoma, invading roots and spinal cord. It is concluded that the degenerative and proliferative lesions represent a continuous series of stages of slowly progressive lesions. The ventral nerve root degeneration (1st stage) is similar to that observed in aging, unirradiated rats, normally developing at the age of 18--20 months.
Examination at 18 months post-term of 139 infants of birth weight less than or equal to 1,500 gm revealed 18 instances (13%) of persistent median nerve damage. All affected infants had received frequent percutaneous brachial artery punctures as neonates. Block sections of the cubital fossa done at autopsy on 12 randomly selected very low-birth-weight infants showed perineural hemorrhage, and Wallerian degeneration or traumatic neuroma of the median nerve in eight patients. It is recommended that brachial artery punctures be avoided whenever possible in the neonatal period.
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