Normative data for a brief neuropsychological battery administered to English- and Spanish-speaking community-dwelling elders

The highly complex and multiple mechanisms responsible for the development of demyelinating neuropathies are reviewed, in particular Guillain-Barré syndrome and its variant Miller Fisher syndrome, chronic inflammatory demyelinating neuropathy, multifocal motor neuropathy, anti-myelin-associated glycoprotein neuropathy, as well as experimental models. Recent investigations into the role of auto antibodies against myelin proteins, or glycolipids have given insights into the pathogenesis of demyelinating inflammatory neuropathies.     

Dermatologic manifestation of Beh?et''s disease

Defects of the peripheral nervous system are common in patients with diabetes mellitus. At least 50% of diabetic patients will develop a form of diabetic neuropathy within 25 years after diagnosis. Currently the cornerstone of treatment lies with the maintenance of euglycaemia using insulin, which has inherent problems of its own. In addition, the signs and symptoms of diabetic neuropathy are often intractable. Therefore, the development of effective treatments for diabetic neuropathy is urgently needed. Thus, animal models have been developed to investigate the pathogenesis of diabetic neuropathy and evaluate potential therapeutic agents. However, no model is perfect and no one would suggest that diabetic rats can replicate the human condition fully. In this review the appropriateness of established animal models of diabetic neuropathy is discussed with reference to the pathology and pathophysiology of the human case with the hope of addresssing some of the questions surrounding this general issue.     

Video-assisted theloscopic electroincision of a high teat stenosis

The hereditary neuropathy Charcot-Marie-Tooth (CMT) type 1A is, in the majority of cases, caused by duplication of the gene for the peripheral myelin protein PMP22, which leads to abnormally increased PMP22 expression. Recent in vitro and in vivo data indicate a novel function of PMP22 in Schwann-cell growth and differentiation other than its role in myelination, and suggest that overproduction of PMP22 leads to a new Schwann-cell phenotype in CMT1A. Taking these data into account, we developed a new hypothesis on the pathogenesis of CMT1A neuropathy: that the defective myelin stability and turnover observed in the disease is caused by altered PMP22 gene dosage and its resultant effect on abnormal Schwann-cell growth and differentiation.     

Degenerative complications of diabetes. Value of systematic Doppler echocardiography

Autonomic neuropathy in diabetes mellitus can cause alterations of the motor function of various segments of the gastroenteric apparatus. With hepatocholangio-cholecystiscintigraphy-HIDA we have studied the motility of the biliary system in patients with diabetes mellitus type II. The research has been carried out in 29 patients with diabetic autonomic neuropathy; 12 healthy volunteers have been studied to compare the results obtained. The results showed a delay in the appearance of radionucleotide in the small intestine of diabetic subjects compared to controls with statistical significance. Moreover the diabetic subjects with a serious neuropathic injury showed increased intestinal transit time. These results match those obtained by other authors that have studied the cholecystic emptying in diabetic subjects with other methods. Consequently the biliary system is also affected by the diabetic autonomic neuropathy that can be in its turn the cause of other pathologies such as biliary lithiasis.     

Metabolic neuropathies

A large epidemiological study has documented that one-third of diabetic patients have peripheral neuropathy. Diabetes duration, poor glycaemic control, smoking and hypertension are all independent predictors of the incidence of diabetic polyneuropathy. High prevalence of autonomic dysfunctions, both sympathetic and parasympathetic, has been found in patients with nonalcoholic chronic liver disease. The pathogenesis of metabolic neuropathy is unclear; even immunologic factors might play a role in the development of diabetic autonomic neuropathy. No specific treatments are available for these neuropathies. Correction of metabolic derangement is fundamental, as shown by the amelioration of peripheral nerve function obtained after successful simultaneous pancreas-kidney transplantation. The therapeuthic potentials of neurotrophins for the prevention and treatment of diabetic neuropathy have to be confirmed in future studies.     

Diabetic neuropathy

Better clinical characteristics and a standardized approach to the definition of neuropathy has enabled us to define more precisely the natural history of diabetic neuropathy. Detailed studies on the pathology and pathogenesis have allowed dissection of important pathogenetic pathways. Effective treatment is currently limited, although a number of new and potentially important therapeutic interventions, including modification of the vascular supply and antioxidant status and growth factors, may prove to be of benefit in preventing damage and also promoting repair of peripheral nerves in human diabetic neuropathy.     

Chronic relapsing brachial plexus neuropathy with persistent conduction block

Idiopathic brachial plexus neuropathy (BPN) is an immune-mediated disorder characterized by an acute onset of painful weakness in one or both upper extremities. The course is usually monophasic with gradual improvement over months; however, occasionally BPN can recur. Electrophysiologic studies suggest the pathogenesis is primarily axonal in the majority of cases. We describe an unusual case of BPN in which the patient had a chronic and relapsing course of painless weakness associated with conduction blocks and other electrophysiologic features of demyelination across the brachial plexus. The patient improved following treatment with intravenous immunoglobulin. The neuropathy falls within the spectrum of chronic inflammatory demyelinating polyneuropathy and multifocal motor neuropathy.     

The blood-brain barrier disruption controversy: does it hold water?

OBJECTIVES: A defect in mitochondrial energy conservation is strongly suggested to be involved in the pathogenesis of Leber's hereditary optic neuropathy (LHON). The authors therefore compared the energy charge in lymphocytes among patients with LHON, their asymptomatic maternal lineages, and normal control subjects. MATERIALS AND METHODS: Blood samples were obtained from 7 patients, 10 asymptomatic maternal relatives, and 16 normal subjects. Molecular analysis confirmed that all had the homoplasmic 11,778 point mutation in the mtDNA of their blood cells. The concentrations of adenosine triphosphate (ATP), diphosphate (ADP), and monophosphate (AMP) were determined by high-performance liquid chromatography. The energy charge was calculated as (ATP + 1/2 ADP)/(ATP + ADP + AMP). RESULTS: The mean energy charges of lymphocytes were 0.871 +/- 0.049 in patients with LHON, 0.884 +/- 0.061 in their asymptomatic maternal relatives, and 0.885 +/- 0.061 in normal controls, respectively. No statistically significant difference was found among the three groups. CONCLUSIONS: Although the study did not find the anticipated change in energy charge in peripheral blood cells, this neither confirms nor rejects the notion that a defect in the mitochondrial oxidative phosphorylation system is involved in the pathogenesis of LHON.     

A study on a new antineural antibody in a case of paraneoplastic sensory neuropathy associated with breast carcinoma

Paraneoplastic sensory neuropathy is a remote effect of cancer, usually associated with small cell lung carcinoma and anti-Hu antibody. This report details the case of a 59 year old woman with a breast carcinoma and a paraneoplastic sensory neuropathy characterised by chronic asymmetric sensory neuropathy. Anti-Hu antibody was not detected in her serum; nor were other known antineuronal antibodies such as anti-Ri and Yo. However, we have found an antineural antibody that reacted to a 106 kDa mouse neural antigen which has not yet been reported. Immunohistochemically, this antineural antibody bound to the posterior grey horn. This finding suggests that this antineural antibody may play an important part in the pathogenesis of the sensory neuropathy of this patient.     

Entrapment neuropathy of the deep peroneal nerve associated with the extensor hallucis brevis

The authors report a case of entrapment neuropathy of the deep peroneal nerve associated with the extensor hallucis brevis. This entrapment neuropathy was found distal to the inferior retinaculum that causes the anterior tarsal tunnel syndrome. Surgical decompression of the deep peroneal nerve that was entrapped by the extensor hallucis brevis relieved the symptoms. This condition, like the anterior tarsal tunnel syndrome, deserves attention.     

Protracted posttraumatic optic disc swelling

BACKGROUND: Optic disc swelling is a rare sequela of blunt ocular trauma. METHODS: The authors examined three young patients who had an unusual post-traumatic optic neuropathy in which protracted swelling of the optic nerve head was the salient clinical feature. RESULTS: Associated choroidal ruptures in two patients suggested a contrecoup mechanism of injury to the optic nerve at its junction with the globe. All patients had partial recovery of vision over months, concurrent with resolution of the nerve head swelling and development of optic disc pallor. CONCLUSION: Despite its protracted course, posttraumatic optic disc swelling appears to be associated with a favorable prognosis for visual recovery.     

Nerve growth factor effects on the song control system of zebra finches

PURPOSE: The authors present the "pattern" visual evoked potentials (VEP) analysis with use of the artificial neural networks (ANN). MATERIALS AND METHODS: The study involved 11 patients with compressive chiasmal optic neuropathy, 20 patients with optic neuritis, 12 patients with anterior ischaemic optic neuropathy, 20 patients with optic nerve atrophy from neuritis, 8 patients with demyelinative neuropathy, 5 patients with oedema optic nerve, 20 healthy persons. The tests of visual evoked potentials were performed with the use of computer system UTAS-E1000. Classification of potentials was made by correlation of outputs of ANN with results of confirmed neuro-ophthalmology conditions. RESULTS: ANN of different architecture were classified correctly in 80-100% of VEP record samples. CONCLUSIONS: The obtained correctness of classification confirms usefulness of VEP analysis as the objective diagnostic method in some neuro-ophthalmological diseases and indicates application of ANN in multifactor analysis.     

Multifocal motor neuropathy

We present a review of the literature on multifocal motor neuropathy (MMN), a rare neurological disorder which has features in common with both chronic inflammatory demyelinating neuropathy and lower motor neuron disease. Clinically, MMN is characterised by slowly progressive asymmetrical limb weakness, usually most prominent in the forearms. Weakness may be associated with muscle wasting, fasciculations and decreased tendon reflexes. Serum anti-GM1 ganglioside antibody titres may be increased. The diagnostic hallmark of MMN is the electrophysiological demonstration of persistent localised motor conduction blocks, with otherwise normal or near-normal motor and sensory conduction velocities. The pathogenesis of MMN has not been elucidated completely. There is, however, substantial evidence for an autoimmune mechanism. Based on the possible involvement of the immune system in the pathogenesis of MMN the therapeutic efficacy of several immunomodulatory drugs has been tested. Treatment of MMN patients with human immunoglobulin or cyclophosphamide is usually followed by a marked improvement of strength. The finding that MMN is a potentially treatable disorder underscores the importance of distinguishing MMN from lower motor neuron disease, for which no effective therapy is currently available.     

More about similarities between Geriatric Depression and Well-Being Scales

The mechanisms of pathogenesis for both idiopathic Parkinson disease and non-arteritic anterior optic ischemic neuropathy are unknown. A study has shown that, in both diseases, there is a higher prevalence of gastrointestinal ulcers than in age- and sex-matched controls or than in the reported rates for the general population. It is proposed that gastric Helicobacter pylori infection may be a cause of both these diseases.     

Visual disturbance by lymphocytic hypophysitis in a non-pregnant woman with systemic lupus erythematosus

The authors present the case of a patient with systemic lupus erythematosus who developed visual disturbance and amenorrhea. Though the clinical and radiological findings resembled those of pituitary adenoma, the patient was finally diagnosed as having lymphocytic hypophysitis after the operation. We briefly describe this relatively rare entity in relation to its autoimmune pathogenesis.     

Does hysteroscopy improve upon the sensitivity of dilatation and curettage in the diagnosis of endometrial hyperplasia or carcinoma?

We report the occurrence of a relapsing, severe predominantly motor neuropathy in a 75-year-old man with an IGM-K M-protein binding to gangliosides GM2, GM3, GM4, GD1a, GT1b and LM1. Motor nerve conduction velocities were slowed with conduction block. A superficial peroneal nerve biopsy specimen revealed segmental demyelination and remyelination. The patient improved after repeated plasma exchanges, and the antibody titer decreased in association with clinical recovery. This IgM M-protein has a unique, previously unreported binding specificity for terminal NeuAcalpha2-3Galbeta- moiety in common to all gangliosides bound by the antibody except GM2. M-proteins with this affinity may be involved in the pathogenesis of this and other cases of motor-dominant demyelinating neuropathy.     

Anorectal sensitivity in patients with obstructed defaecation

Patients with obstructed defaecation (OD) perform major defaecatory efforts that lead progressively to pudendal motor neuropathy. Anorectal sensory function in these patients and its possible influence in the pathogenesis of the disease have been little studied. In the present paper we investigated anorectal sensitivity to electric and thermal stimuli in patients with OD, and studied the possible existence of pudendal sensory neuropathy associated to their known pudendal motor neuropathy. Forty subjects were divided into two groups: 21 healthy controls (11 females and 10 males; mean age 51.8 +/- 11 years, range 33-67) and 19 patients with OD (18 females and 1 male; mean age 48 +/- 15 years, range 20-71). The patients with OD suffered constipation and an obstruction sensation upon defaecating, even in the case of soft stools. Clinical perineometry, manometry, pudendal motor latency studies, external anal sphincter single fibre electromyography and the evaluation of sensitivity to electric and thermal stimuli were carried out in all cases. All pudendal motor function parameters showed statistically significant differences between the two groups. In the controls the electrical sensitivity threshold was minimal in the mid anal canal, where sensory receptor presence is greater. Sensitivity was significantly higher in the upper and lower anal canal regions (P < 0.05), and much higher in the rectum (P < 0.001). A similar sensory profile was recorded in the patients with OD, though with significantly higher thresholds at all points with respect to the controls. The thermal stimulus thresholds in the lower and middle anal canal were significantly smaller than in the upper canal region and rectum, and the thresholds were again higher among the patients with OD than among the controls. In all cases the thresholds for heat were lower than for cold stimuli. In both groups the motor function parameters were correlated with the sensory function variables, and the latter between themselves. Patients with OD presented sensory deterioration at all points studied in the anal canal and rectum. Sensory pudendal neuropathy was found to be associated with the pudental motor neuropathy.     

Etiopathology of Beh?et''s disease: herpes simplex virus infection and animal model

Although the detailed pathogenesis of diabetic polyneuropathy is not known, several mechanisms appear to be involved and may occur sequentially. Hence, the early and much researched activation of the polyol-pathway appears to secondarily affect nonenzymatic glycation, perturbation of vasoactive substances, the immune system and neurotrophism. These metabolic abnormalities may be differentially expressed in the neuropathy occurring in insulin dependent diabetes mellitus (IDDM) and non-insulin dependent diabetes mellitus (NIDDM) diabetes. This notion is supported by differences in the structural abnormalities of the neuropathies in the two types of diabetes. Distinct and characteristic nodal changes occur in IDDM but not in NIDDM neuropathy, which also shows a milder axonal atrophy. On the other hand, nerve fiber loss which characterizes diabetic neuropathy tends to be focal in the older NIDDM patients, suggesting a more prominent vascular genesis. A further characteristic feature of diabetic neuropathy is blunted fiber regeneration, which probably is consequent to impairments of the necessary immune response and local synthesis of neurotrophic factors. Nerve biopsies from diabetic patients, although not necessary for diagnosis, provide valuable tissue for biochemical and molecular analysis of underlying mechanisms, the detailed elucidation of which will facilitate the design of targeted therapies.     

Spontaneous dissection of cerebral arteries. I. Carotid arteries and their branches

Artery dissection means tearing apart of its layers by blood coming inside after endothelial damage. The authors describe the prevalence of that pathology of carotid, arteries (diagnosed rarely so far), pointing to early onset age and to its multifactorial aetiology and pathogenesis. They also outline the pathomechanism of the neurological symptoms and emphasize the variety of the clinical manifestations. The diagnostic possibilities of artery dissection are also presented.     

Bacterial epidemiology of chronic otitis. Prophylactic and therapeutic deductions

The early onset sensory motor hereditary neuropathy (HSMN) can be divided into two forms: the early onset type (HSMN type III or Dejerine-Sottas) and the congenital hypomyelinating neuropathy (CHN). In both cases, abnormalities of myelination are present in peripheral nerves. Symptoms include hypotonia, weakness, hypotrophy, and areflexia. Skeletal changes may be present. In CHN symptoms may be present at birth and are rapidly progressive. Many authors actually consider the two forms different. The diagnosis is based only on clinical and neuropathological criteria. Here we report a case with a typical phenotype of HSMN type III but with peripheral nerve bioptic findings suggesting a CHN.