Chronic Acquired Demyelinating Polyneuropathy following Renal Transplantation.

The clinical, laboratory, and treatment findings of a patient with chronic acquired demyelinating polyneuropathy (CADP) in association with renal transplantation are described. Like the present case, many such patients have been described under the rubric of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).

Therapy in neuromuscular disease.

The therapy of myasthenia gravis and inflammatory myopathy are discussed in detail in this article. The discussion of these two disorders illustrates the extraordinary progress that has been achieved in the therapy of neuromuscular disease.

Myopathy with tubulin-reactive crystalline inclusions.

A 61-year-old man with muscle aches and persistently elevated serum creatine kinase had aggregates of randomly oriented, rhomboidal or rectangular protein crystalline inclusions in the sarcoplasm of type II fibers. Immunochemical studies showed strong reactivity of the inclusions to tubulin antibodies, suggesting that these unique crystalline inclusions may be a consequence of altered synthesis, processing, or degradation of tubulin.

Medical therapies in myasthenia gravis.

Forty years ago, a patient with MG probably had a fifty-fifty chance of surviving a myasthenic crisis, defined as the need for mechanical ventilatory support. Approximately 16% of all patients experience a crisis, a figure that has not changed appreciably since then. Progressive weakness, oropharyngeal symptoms, refractoriness to anticholinesterase medication, intercurrent infection, and invasive procedures including needle biopsies of thymic gland masses, and reactions to contrast agents used in the performance of CT of the chest have been implicated in the development of crisis. It is now standard practice to treat severe crisis in an intensive care unit. The ready availability of intensive care in most hospitals belies the fall in the mortality of myasthenic crisis to 6% over the past several decades. Crisis is a temporary exacerbation, regardless of the proximate cause, and the goal is to keep the patient alive until it subsides, usually in 2 weeks. In the past, edrophonium was used to differentiate myasthenic crisis from cholinergic crisis, but that is now moot because withdrawal of cholinesterase medication is necessary for improvement in both situations. The underlying immunologic derangements in myasthenic crisis are not well understood, but there is a rapidly fatal antibody-mediated syndrome that bears resemblance to crisis and is associated with inflammation and necrosis of the end-plate region.

Person-centred care for people with dementia: a quality audit approach.

This paper addresses the concept of person-centred care for people with dementia by consideration of an audit process using dementia care mapping as the audit tool. It is argued that this tool is best for identifying the lived experiences of the people in receipt of care. As a result it is able to identify the overall culture of care and its level of 'person-centred' approach. The audit was conducted on 12 units, half of which were day units and the others catering for inpatients. Five patients were mapped on each day for a 4-day period. The results give some idea of the quality of care and identify where improvement is necessary. Scores such as well-being values and the Dementia Care Index give clear signposts to the level of person-centred care and highlight where staff development is necessary. Recommendations are given to aid on-going planning.

Dementia care mapping: an approach to quality audit of services for people with dementia in two health districts.

The audit reported in this paper and submitted to the Psychiatry of Old Age Management group, assessed six units within each of two health districts in the UK. Using a nonparticipatory observation method in the units selected, the aim was to measure quality and the environment of care. Dependency levels of the clients/residents were also estimated to give a clearer picture of the setting and the care requirements. This was intended to establish a baseline for the units mapped and to enable care developments to be focussed upon intended outcomes. Results led to a number of observations related to the levels of interaction between staff and clients/residents, the need for a wider range of activities to promote person-centred care, and a suggested route to the improvement in quality of life for this vulnerable group of people. Assessment of dependency levels linked to the results of the mapping showed that high dependency does not lead automatically to a lower quality of person centred care.

Anti oppressive practice: a route to the empowerment of people with dementia through communication and choice.

This short study looks at the issue of anti oppressive practice and the way that it relates to the care of people with dementia. In particular it considers ways in which people can be empowered and given choices around aspects of day-to-day living in a care setting. The study took place over a period of 6 months and utilized the Dementia Care Mapping (DCM) observation process to assess the level of well being of residents in the setting studied. The results can only be seen as a first stage in the process of understanding ways in which people with dementia may be empowered. It is demonstrated that a change of practice developed over the 6 months between the two observation periods. Areas such as communications and day-to-day activities are examined to identify both problems and strategies for such change. The conclusion highlights the need to continue the mapping exercise as a way of ensuring that change is not only maintained but also advanced.

Local activation of the complement system in endoneurial microvessels of diabetic neuropathy.

Quantitative immunocytochemical analysis of complement proteins (CP) was performed on sural nerve biopsies from 15 patients with diabetic neuropathy (DN) and 18 nondiabetic patients with other forms of chronic neuropathy (ON). The mean age of the patients and the pathological severity of the neuropathy were similar in both groups. The percentage of patients that expressed strongly immunoreactive CP in the walls of endoneurial microvessels was significantly greater in DN than in ON for all proteins tested. C3d neoantigen was expressed in 100% of DN cases compared with 17% of ON; and membrane attack complex (MAC), C5b-9 neoantigen, in 93% of DN and 17% of ON. In the cases with DN, 81% of endoneurial microvessels, as identified by the endothelial marker, Ulex europaeus, contained C5b-9 neoantigen deposits, compared with 22% in those of ON, and the staining in DN was significantly more intense. Expression of the neoantigens of C3d and C5b-9 in nerve implies local activation of the complement system. In DN, activation of the complement pathway and formation of the MAC could injure blood vessels and adversely affect the circulation in the endoneurium.

Diabetic peripheral neuropathy.

Diabetes mellitus leads to several recognizable clinicopathologic neuropathic syndromes. Diagnosis and evaluation requires a thorough history and neurologic examination, nerve conductions and needle electromyography (EMG), blood studies, consideration of cerebrospinal fluid analysis, and nerve and muscle biopsy in the most severely affected patients. Microangiopathy is the commonest cause of diabetic neuropathy, associated with potentially reversible metabolic, immunologic, or ischemic injury. Tight glycemic control and symptomatic therapy is beneficial in some patients but does not prevent progression of neuropathy especially in patients with severe motor and gait disability. Intravenous immune globulin is a novel therapy in diabetic patients. It may be considered in selected patients well characterized by clinical, electrophysiologic, histopathologic studies, and one of the following progressive syndromes: mononeuropathy multiplex, primary demyelinating motor or sensorimotor neuropathy, and peripheral nerve perivasculitis or microvasculitis associated with vascular membrane attack complex protein deposits.

Selegiline is ineffective in a collaborative double-blind, placebo-controlled trial for treatment of amyotrophic lateral sclerosis.

BACKGROUND: The cause of amyotrophic lateral sclerosis (ALS) is not known, and there is no effective treatment. Cell death may be caused by oxidative damage. Selegiline hydrochloride (Eldepryl) is a monoamine oxidase-B inhibitor with antioxidant properties. OBJECTIVE: To determine if selegiline affects the clinical course of patients with ALS. DESIGN: Six-month, double-blind, placebo-controlled study of 133 patients with classical ALS and symptoms for less than 3 years. The primary end point to indicate effectiveness was the rate of change of the Appel ALS total score, an index of disease severity that incorporates strength and function in limbs, respiratory function, and bulbar function. RESULTS: Of the 133 patients, 67 were randomized to receive selegiline and 66 to receive placebo. One hundred four patients (53 in the selegiline group and 51 in the placebo group) completed the 6-month trial. Both groups were comparable for baseline characteristics and mean Appel ALS total score (70.5 points for the selegiline group and 70.6 for the placebo group). There was no difference in the rate of progression as measured by the Appel ALS total score, showing an average increase of 22 points in 6 months. The monthly rate of change was 3.4 for the selegiline group and 3.5 for the placebo group. There was 1 adverse reaction: worsening depression. Seven patients died during the study (4 in the selegiline group and 3 in the placebo group). CONCLUSION: Selegiline treatment had no significant effect on the rate of clinical progression or outcome of ALS.

Diabetic peripheral neuropathy.

Diabetes mellitus leads to several recognizable clinicopathologic neuropathic syndromes. Diagnosis and evaluation requires a thorough history and neurologic examination, electrophysiologic studies, blood studies and, in selected cases, cerebrospinal fluid analysis and nerve and muscle biopsy. Microangiopathy is the leading cause of diabetic neuropathy associated with metabolic, vascular ischemic and immunologic injury. Tight glycemic control and symptomatic therapy is beneficial in a minority of patients but does not prevent the relentless progression of symptoms and signs. Intravenous immune globulin is a novel therapy in patients with mononeuropathy multiplex, primary demyelinating neuropathy and peripheral nerve T-cells microvasculitis associated with C5b-9 membrane attack complex protein deposits.

Lyme neuroborreliosis.

The series on treatable neuromuscular disorders continues with the present article on the peripheral nervous system manifestations of Lyme neuroborreliosis.

Lymphoproliferative disorders and motor neuron disease: an update.

We studied 26 patients with both motor neuron disease and lymphoproliferative disease (LPD). Twenty-three patients had definite or probable upper motor neuron signs; none had electrophysiologic evidence of motor neuropathy. LPD syndromes comprised Waldenström's macroglobulinemia, multiple myeloma, chronic lymphocytic leukemia, follicular cell lymphoma, and Hodgkin's disease. In all but one patient, the cause of disability or death was neurologic. LPD was confined to bone marrow in 14 patients; eight of 14 had monoclonal paraproteinemia. One patient had LPD discovered at autopsy. Treatment of LPD in 20 patients resulted in neurologic improvement in 1 patient and arrest in another; both had progressive spinal muscular atrophy. Eleven patients were worse and 13 died. At least 30 cases have been reported from other centers, bringing the total to 56. Among the unusual reported concomitants were POEMS (polyneuropathy, organomegaly, endocrinopathy, myeloma, and skin changes) syndrome of myeloma and angiotropic lymphoma.

Vasculitis and the nervous system. Historical perspective and overview.

This article is an introduction to the historical background, clinical and laboratory diagnosis, pathogenesis, and treatment of vasculitis involving the peripheral and central nervous system. It also provides a background for the articles that follow.

Radiographic features of central nervous system vasculitis.

Central nervous system (CNS) vasculitis refers to primary and secondary disorders of the CNS vasculature. Most authorities agree that CNS vasculitis is a potentially serious disorder; therefore, prompt diagnosis and initiation of therapy are high priorities in treatment. Remarkable progress has been made in the diagnosis, evaluation, and treatment of this disorder. This article examines many aspects of the radiographic evaluation of CNS vasculitis.

Granulomatous angiitis of the nervous system.

Granulomatous angiitis of the nervous system (GANS) refers to distinctive clinicopathologic disorders with the essential feature of granulomatous inflammation of cerebral and spinal vessels, accompanied by multinucleate giant cells and epithelioid cells. This article reviews and examines the clinical, laboratory, and neuropathologic findings of patients with granulomatous angiitis.