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[Neurological manifestations in monoclonal gammapathies. Pure neurological manifestations. Immunofluorescence study].

Analysis of 105 peripheral and central nervous system complications in 1062 monoclonal gammapathies draws attention to two types of phenomena. The possibility of pure neurological manifestations of IgM monoclonal gammapathies with macroglobulinorachia leads to discussion of their nosological position in relation to Waldenström's disease, Burkitt's lymphoma and Marek's disease. It is suggested that these cases should be reclassified under the heading "secreting neurolymphomatosis". Immunofluorescence and electron microscopy of 10 biopsies of the peripheral nerve showed deposits of monoclonal immunoglobulin whose function in determining peripheral neuropathies is discussed. The simultaneous presence of lymphoid infiltration, amyloid deposits and the monoclonal immunoglobulin (M component) suggests that this immunoglobulin could be the link between the cellular infiltrate secreting it and amyloid infiltration which would be the visible manifestation of it.

Amyotrophic Lateral Sclerosis

Neurological manifestations of gnathostomiasis.

Neurological features of 24 patients with nervous system gnathostomiasis were reviewed. The commonest presenting features were radiculomyelitis or radiculomyelitis terminating with encephalitis, and subarachnoid haemorrhage. A primary encephalitic form was noted in 2 patients. The salient feature of the disease was a sudden onset of extremely severe radicular pain and/or headache followed by paralysis of the extremities and/or the cranial nerves. Migration signs were also the hallmark of nervous system gnathostomiasis. No single area of the nervous system was inaccessible to the highly invasive gnathostome lava. Multiplicity and/or rapid progress of lesions beyond the degree of cerebral oedema could only be explained by further migration of the parasite. Death occurred in 6 patients from direct extensive involvement of vital centres in the brain stem or from complications such as pneumonia or septicaemia. Multiple cranial nerve palsies were usually bad prognostic signs.

Adolescent

The neurological manifestations of chronic inhalation of leaded gasoline.

Abnormal neurological signs were found in 46 of 50 children and adolescents chronically sniffing leaded gasoline. These abnormalities resolved within eight weeks in all but one case. Exaggerated deep reflexes, postural tremor and evidence of cerebellar dysfunction occurred in a highly significnat number of patients. Forty-nine had blood lead levels greater than or equal to 40ug/dl. The mean blood lead levels were significantly higher in those with (a) abnormally brisk deep reflexes and (b) with evidence of cerebellar dysfunction, than in those without these findings. Five optional treatment regimes were employed and a classification was used, based on clinical findings, initial blood lead levels and the response to the calcium disodium edatate mobilization test. 39 patients received chelation therapy. These data suggest that neurological manifestations occur frequently in those abusing leaded gasoline and that chelation therapy has an important place in their management.

Adolescent

Neurological manifestations of Paget's disease.

The neurological features of 96 patients whose skull and spinal X-rays showed the features of Paget's disease of bone were reviewed. The clinical phenomena were compared with those in another group of patients, identified by having a normal skull X-ray. Cranial nerve lesions and spinal syndromes could be attributed to the Paget's disease in many cases but the evidence did not suggest that other clinical phenomena such as dementia and epilepsy were related to the bony changes. Amongst cranial nerve lesions deafness is certainly attributable to Paget's disease, hemifacial spasm when combined with other signs is probably related, and possibly also trigeminal neuralgia. Basilar invagination can be asymptomatic, but deafness, hemifacial spasm and pyramidal signs were more prevalent in its presence. The importance of reviewing the cause and effect relationship between Paget's disease and neurological problems is discussed in the context of the therapeutic use of calcitonin.

Aged

The neurological manifestations of porphyria: a review.

The hereditary hepatic porphyrias, PV, AIP and HC, are characterized biochemically by increased excretion of porphyrins and the porphyrin precursors ALA and PBG. They are characterized clinically by episodes of acute neurological involvement. The increased production of porphyrins and porphyrin precursors has been shown to be due to partial enzyme blocks along the heme biosynthetic pathway which results in secondary depression of the key enzyme ALA-synthetase. The neurological manifestations could therefore be related to either a decrease in essential heme-proteins or other heme-containing compounds within the nervous system, or to a toxic effect of the over-production of the porphyrin precursors ALA and PBG. There is evidence for and against both theories. Recent work from a number of research groups has shown the porphyrin precursors to have potent pharmacological effects on the nervous system, and these are possibly related to the GABA receptor and binding site-porphyrin precursor interactions. Current studies on therapy of the acute attack have concentrated on suppression of ALA-synthetase activity, and consequently, on reduced ALA and PBG production. A number of such methods of therapy have met with remarkable success and hold promise for the future treatment of the acute attack.

Acute Disease

[Case of orthostatic arterial hypotension with neurologic manifestations (Shy-Drager syndrome)].

The authors report a case of idiopathic orthostatic arterial hypotension with neurological manifestations, rarely diagnosed in this country. The clinical manifestations corresponded to those of Shy-Drager syndrome, with an akinetic-hypertonic syndrome, pyramidal signs, sphincter disturbances, sexual potency loss and neurogenic muscle atrophy. Attention is called to the recent results of biochemical investigations of the blood in this syndrome, and among them to the blood level of noradrenaline which may be useful in the diagnosis.

Fecal Incontinence

Os odontoideum in children: neurological manifestations and surgical management.

The os odontoideum is a congenital anomaly of the 2nd cervical vertebra. It is an ossicle that is separated from the body of the axis by a variable transverse gap. This weakens atlantoaxial stability. When ligamentous support also fails, subluxation ensues. Compressive myelopathy and vertebral artery obliteration may occur. Eight cases, all in children, were studied prospectively. The neurological manifestations and surgical indications and treatment are discussed.

Adolescent

Cranial computed tomography findings in xeroderma pigmentosum with neurologic manifestations (De Sanctis-Cacchione syndrome).

Computed tomography (CT) scans in two young patients with xeroderma pigmentosum with neurologic manifestations (De Sanctis-Cacchione syndrome) showed ventricular dilatation and cerebral cortical atrophy. The brainstem appeared small. In addition, an abnormal thickening of the calvarial bones was noted in both patients. These CT findings of the brain were compared with the neuropathologic features of this syndrome reported in the literature.

Adolescent

[Neurologic manifestations of periodic disease].

Results of comprehensive neurological examinations of 4 patients suffering from periodic disease have shown that the latter may be not only a disease entity, but also a syndrome characterizing some organic affections of the brain. The abdominal and other crises of the periodic disease included cerebrovascular disturbances which manifested hemipareses, syncopes, and migraine-like cephalgia. EEG and REG examinations have revealed dysfunction of the mesodiencephalic structures which, probably, underlies the pathogenesis of the periodic disease. The knowledge of the formerly almost unexplored neurological manifestations of the periodic disease will contribute to improvement of the therapy of this rare ailment.

Abdomen

Study of blood pyruvic acid levels in pellagrins with and without neurological manifestations and response to nicotinic acid therapy.

In 25 cases of pellagra and 10 healthy controls, the blood pyruvic acid levels in the fasting stage and after 60 and 90 minutes of glucose load were estimated by the technique of Friedmann & Haugen (1943). The blood pyruvic acid levels after 60 and 90 minutes of glucose load were significantly higher in pellagrins as compared to controls. The following conclusions were drawn from this study: (i) that there is impairment of pyruvic acid metabolism in cases of pellagra, which is more marked in pellagrins with neurological manifestations than in those without; (ii) that after administration of nicotinic acid alone for 15 days the pyruvic acid levels returned to normal, thereby indicating that nicotinic acid deficiency is the cause of deranged pyruvate metabolism; (iii) that there is significant improvement in neurological status after nicotinic acid therapy.

Adolescent

[Neurologic manifestations in Mycoplasma pneumoniae. Apropos of 12 cases].

Specific serological methods now enable us to demonstrate that Mycoplasma Pneumoniae is responsible for a variety of neurological symptoms. We describe 12 new cases: 2 encephalites, 8 diffuse polyradiculoneurites and 2 isolated lesions of the cranial nerves. These 12 cases, together with the 42 recorded in the literature, bring total number of published cases to 54. Analysis emphasizes the extreme polymorphism of the neurological manifestations and their favourable prognosis in most cases, however serious the initial symptoms. Although the pathogenesis of neurological symptoms produced by Mycoplasma Pneumoniae is still debatable, evidence supports an immunoallergic reaction.

Adolescent

Neurological manifestations of rhabdomyosarcomas in the head and neck in childhood. Neuroradiological study.

Two cases of rhabdomyosarcoma in the head and neck with neurological manifestations are presented. Brain scan demonstrated an apparent area of increased uptake of 99mTc in the tumor. Cerebral angiography revealed a sharply demarcated but rather faint tumor stain. The contributing vessels were hypertrophied branches of the external carotid artery, a dural branch of the internal carotid artery, and branches of the vetebrobasilar systes emphasized because the recent combination of chemotherapy in conjuction with surgery and irradiation seems to be encouraging.

Adolescent

Variegate porphyria with unusual neurologic manifestations.

A 62-year-old man with variegate porphyria is reported. This is the first case to be described in Greece. Apart from the common features of the disease this patient exhibited sensory loss of the syringomyelic type. Though variegate prophyria with neurologic manifestations is not uncommon syringomyelic type of sensory loss is most unusual.

Diagnosis, Differential

Neurologic manifestations of diabetic comas: correlation with biochemical alterations in the brain.

Coma and other neurologic abnormalities are present in patients with either diabetic ketoacidosis (DKA) or nonketotic coma (NKC), and the cause of such phenomena are not known. Patients with NKC also manifest seizures and focal neurologic changes. Treatment of diabetic coma with insulin may induce cerebral edema by as yet undefined mechanism(s). In patients with DKA, cerebral oxygen utilization is impaired, and there is hyperviscosity of the blood. A substantial part of the brain's energy source is derived from ketones, which in themselves can depress sensorium. Extracellular hyperosomolality is present, which may also contribute to the genesis of coma. In addition, most ketoacidotic patients have associated medical conditions, which may further impair consciousness. Biochemical changes in the brains of animals with DKA include impairment of both phosphofructokinase activity and pyruvate oxidation, and accumulation of citrate. The net effect upon sensorium in ketoacidotic patients probably represents the interaction of most of the above factors and differs markedly among individuals. Patients with NKC manifest not only depression of sensorium, but also focal motor seizures, hemiparesis, and other neurologic changes, such as aphasia, hypereflexia, sensory defects, autonomic changes, and brainstem dysfunction. Most of the aforementioned changes revert to normal after correction of hyperosomolality. Gamma amino butyric acid, which has been shown to elevate the seizure threshold, is normal in brains of ketoacidotic animals, but may be low in nonketotic coma. Also, hyperosomolality per se may produce seizures. Cerebral edema may complicate the treatment of either DKA or NKC. The available experimental evidence suggests that many of the commonly held theories for the production of such brain swelling probably do not occur. There is no breakdown of the sodium pump, sorbitol or fructose do not accumulate in brain, and brain glucose is only about 25 percent of that in plasma; Cerebral edema is probably produced largely by a direct action of insulin on brain at a time when plasma glucose is approaching normal values. Cerebral edema can thus theoretically be avoided by stopping insulin when plasma glucose has been lowered to values approaching normal.

Animals

[Study of neurological manifestations of chronic lymphatic leukemia (lymphosis). Role of associated globulin anomalies (apropos of 8 cases)].

The authors emphasize the relative frequency of neurological symptoms in lymphatic leukaemia. Clinically, these cause more or less diffuse encephalitic or multineuritic syndromes, generally a combination of the two. Their pathogenesis is usually connected with lymphoid tissue infiltration into either the meninges or the vascular sheaths of the central nervous system or the sheaths of the roots or of the peripheral nerves. The authors stress the possible function of immunoglobulin abnormalities of the C.S.F. indicative of the presence of the leukaemic process within the nervous system. This pathogenesis prompts the use of therapeutic methods directly attacking leukaemic infiltration of the nervous system (focal cobalt therapy, intrathecal chemotherapy) and the authors have found that these give favourable results.

Aged