Multiple Sclerosis impact on employment and income in New Zealand.

BACKGROUND AND OBJECTIVES: We investigated the demographic, social and clinical characteristics associated with employment status and income for people with multiple sclerosis (MS) in New Zealand (NZ). METHODS: The NZ National MS Prevalence study included all persons resident in NZ on census day 2006 diagnosed with MS (96.7% coverage). Factors associated with employment and income status among the working age population (25-64 years) were identified by linear regression. RESULTS: Over 90% of working age people with MS (n=1727) had a work history, but 54% were not working. Work loss occurred early in the disease course, and at low disability (P<.001). Advancing age, progressive disease, longer disease duration, higher disability levels, partner loss and lower education were associated with work loss (P<.001). Working age people with MS had lower income than the NZ population (P<.0001). Higher qualifications yielded no additional income for MS females and about half the additional income for MS males (P<.0001). CONCLUSIONS: MS profoundly reduces employment and income early in the disease course, and at low levels of disability, however, unemployment is not entirely accounted for by clinical, social and demographic factors. These findings suggest social supports should be explored early in the disease course to reduce loss of income and unemployment for people with MS.

Scales for rating impairment in multiple sclerosis: a critique.

We reviewed scales proposed over the past 35 years for the rating of neurologic impairment in MS. We focused on the Expanded Disability Status Scale (EDSS) which has been recommended as the most useful scale for rating impairment by the International Federation of Multiple Sclerosis Societies in its Minimal Record of Disability for Multiple Sclerosis. We consider that the EDSS has important flaws that seriously limit its usefulness. In light of the widely accepted three-tier classification of dysfunction developed by the World Health Organization, the title is inappropriate. More substantial problems include inadequate precision in defining the degree of impairment in some functional categories of the scale, and the use of a mixture of neurologic signs elicited on examination and subjective information obtained from the patient in defining the overall scale. We suggest guidelines for developing a "Neurologic Impairment Scale" to rate impairment in MS.

The indirect assay for leukocyte migration inhibitory factor (LIF)--standardization and the effect of pH.

In the indirect assay for leukocyte migration inhibitory factor (LIF), lymphokine-rich supernatants were obtained by culture of stimulated lymphocytes and then tested for LIF activity in agarose plates using purified granulocytes as target cells. Studies on the standardization of the conditions under which LIF acts on the target cells are described, with emphasis on the use of "standard" supernatants of known LIF activity and the influence of pH on the action of LIF and the sensitivity of the assay. The observation that LIF activity is reduced when the ambient pH falls below 7.2 is suggested as an explanation firstly for the "escape" phenomenon seen particularly in capillary tube assays for LIF, and secondly for the reduced sensitivity of the capillarly tube assay in comparison with the corresponding agarose plate assay.

Measurement of cerebrospinal fluid IgG in the diagnosis of multiple sclerosis.

CSF IgG was measured in 190 patients by Single Radial Immunodiffusion, A high value for the ratio CSF IgG/total protein (normal values being up to 14%) was obtained in 13 of 22 patients (54%) with definite or probable multiple sclerosis and in 16 of 137 patients (12%) with other neurological disorders excluding acute infections, subarachnoid haemorrhage and neurosyphilis. In 56 patients IgG and albumin were measured in CSF and serum using Rocket Immunoelectrophoresis (Electroimmunodiffusion) and results assessed by dividing the radio IgG/albumin in the CSF by the same ratio in the serum to obtain an Index. Because of the small number of normal patients, it was not possible to define a normal range but separation of patients with multiple sclerosis from those with other neurological disorders was even less clear-cut than with first method. Minor technical difficulties with the Rocket technique and prolonged storage of some of the specimens may at least partly explain the discouraging results with this method. It was concluded that the measurement of CSF IgG was of limited value to a clinican attempting to make a diagnosis in an individual patient with neurological disease.

Infarction of the conus medullaris.

We describe 5 patients who presented with an acute cauda equina syndrome, which we believe was due to infarction of the conus medullaris. In 3 patients, the onset was spontaneous, and in 2 patients it was secondary to temporary occlusion of the distal aorta during medical manipulation. Pain in the buttocks and posterior thighs was a prominent initial symptom in the 3 patients with unprovoked attacks. The main clinical features were profound impairment of bowel and bladder function and of perianal and perineal sensation (S3 to S5 segments). There was sensory and motor impairment in the legs of variable extent, most marked in the S1 and S2 segments, but extending as high as L4 in 2 patients. In 1 patient, ischemic changes in the conus medullaris were confirmed post mortem. Ischemia confined to the caudal tip of the spinal cord is rare, and an underlying anomaly of the pattern of arterial supply is a likely predisposing factor. Four patients had partial return of function over a period of weeks.

Chronic meningitis without predisposing illness--a review of 83 cases.

Eighty-three previously healthy patients with chronic meningitis presenting to a general hospital were reviewed. The single most common cause was tuberculosis (40 per cent) with cryptococcosis (7 per cent), malignancy (8 per cent) and other definable causes much less frequent. In one-third of patients (34 per cent) no cause was found although most appeared to respond to treatment either with anti-tuberculosis drugs or steroids. There were some differences in clinical presentation, the clinical picture being most characteristic in tuberculous meningitis and malignant meningitis. However, there were frequent exceptions to any general rule and, particularly in adults, the clinical features and changes in cell count, protein and glucose in the cerebrospinal fluid (CSF) did not reliably distinguish one form of chronic meningitis from another. Cerebral and meningeal biopsy produced disappointing results with respect to practical guidance in management. It is often necessary to begin treatment before a cause has been identified. Important points in management include the early use of anti-tuberculosis treatment and the difficulty in deciding on a trial of treatment with corticosteroids or amphotericin B. Not emphasised in previous reports is a subgroup of patients with idiopathic chronic meningitis who are responsive to treatment with steroids. Those patients tend to follow a more prolonged indolent course and often require long-term immunosuppression to control symptoms.

The crossed upgoing toe sign: a clinical study.

We compared the crossed upgoing toe sign with the plantar response as an indicator of pyramidal tract dysfunction in 125 normal subjects and 192 patients with neurological disorders. A positive crossed upgoing toe sign was associated significantly with a partial pyramidal tract lesion in the contralateral cerebral hemisphere with a frequency similar to that of Babinski's sign. Unlike Babinski's sign, however, the positive crossed upgoing toe sign was lost when pyramidal weakness was severe enough to produce paralysis of voluntary dorsiflexion of the great toe, and it was found only rarely with pyramidal tract lesions of the spinal cord. The crossed upgoing toe sign has little value as a sensitive indicator of a pyramidal tract lesion. It is potentially of limited value as an aid in determining the level of a pyramidal tract lesion (cerebral hemisphere versus spinal cord), but its usefulness is seriously impaired by the high frequency of false positive signs in normal subjects and patients with other neurological disorders.

A sensitive silver stain for proteins in agarose gels.

A silver stain for proteins in agarose gels which is at least 10 times as sensitive as Coomassie blue is described. The method is simple to use and is particularly useful for the study of protein bands in the gamma region on electrophoresis of fluids such as cerebrospinal fluid in which the protein concentration is low. It readily detects bands of IgG containing 20 to 40 ng/band (approx 3 to 6 ng of IgG/mm2 of gel).

Lower cranial nerve motor function in unilateral vascular lesions of the cerebral hemisphere.

Motor function subserved by cranial nerves V, VII, X, XI, and XII was assessed in 100 patients with hemiparesis due to a unilateral vascular lesion of the cerebral hemisphere. Several of the findings were not described clearly in many of the standard textbooks of neurology. Weakness of sternomastoid when present was always contralateral to the hemiparesis. This emphasises the principle that the cerebral hemisphere controls movement of the body parts in or towards the contralateral half of the body rather than simply the contralateral muscle groups. An apparent exception to this was seen, however, in the small group of patients who had unilateral weakness of the tongue. In those patients deviation of the tongue was towards the hemiparetic side--that is, the cerebral hemisphere controlled the contralateral half of the tongue and hence protrusion towards the ipsilateral side. Mild dysarthria was common with both right and left sided hemiparesis.

The natural history and the influence of surgical treatment in syringomyelia.

A retrospective analysis was made of the natural history and treatment of 44 patients with syringomyelia, followed for a median interval of 10 years after diagnosis. Twenty-four patients were treated surgically. Four of 12 patients who had a laminectomy with aspiration of the syrinx or syringostomy, and 7 of 15 patients who underwent decompression and exploration of the cervicomedullary junction, showed sustained improvement or stabilisation of their neurological deficits. When symptoms had been present for more than 2 years, when there was a moderate or severe neurological disability, and when the patient was more than 40 years old, the long-term outlook of posterior fossa surgery was usually poor. Of the 20 patients who did not have an operation, 7 had no further progression in symptoms after presentation and prolonged survival was usual. It is concluded that in many cases syringomyelia is compatible with prolonged survival, and although improvement can follow surgery, its influence on the disease in the long-term is uncertain.

The natural history of syringomyelia.

A retrospective analysis was made of the clinical course in 24 patients with syringomyelia who had not been treated surgically. 5 patients died during follow-up, and the survivors were followed for 1 to 37 (median 9) years after diagnosis. 8 of the 19 survivors followed a slowly progressive course over 4 to 46 (median 27) years but only 3 were severely disabled. 3 patients had an intermittent progression of symptoms, often with long intervening periods of stability. 8 patients had no progression in symptoms after presentation; none of these patients was severely disabled at last follow-up. It is concluded that the natural history of syringomyelia is unpredictable. Some patients have no further progression in symptoms for many years after onset. Prolonged survival is usual even among those patients with a slowly progressive deficit. The uncertain progression of the clinical course presents considerable difficulties in the timing of surgical intervention and in interpretation of the results.

Leptomeningeal and brain biopsy in chronic meningitis.

BACKGROUND: The cause is not found in one third of patients presenting with chronic meningitis. Biopsy of the leptomeninges and brain is often recommended in these patients, but the value of a biopsy is uncertain. AIMS: To review the results of leptomeningeal and brain biopsies and their effect on diagnosis and management in patients with chronic meningitis, if the diagnosis was uncertain after clinical assessment, lumbar puncture and radiological investigations. METHODS: The clinical features, investigations and the results of leptomeningeal and brain biopsies were reviewed retrospectively in 25 patients presenting with chronic meningitis between 1967 and 1990. RESULTS: The biopsy identified the cause of the chronic meningitis in five patients (neoplastic meningitis in three, tuberculous meningitis in one, granulomatous angiitis in one). In 17 patients the biopsy was abnormal but it did not identify the cause (non-granulomatous lymphocytic meningitis in eight, granulomatous meningitis in two, non-specific abnormalities in seven). In two patients the biopsy was normal and in one patient the tissue was used only for culture. The results of the biopsy led to a beneficial change in treatment in two patients but did not influence management in the other patients. CONCLUSIONS: A leptomeningeal and brain biopsy was of limited practical value in diagnosis and management in most patients with chronic meningitis.

Prostaglandin effect on lymphokine production in multiple sclerosis.

Production of the lymphokine LIF (leukocyte migration inhibitory factor) by normal lymphocytes on stimulation with mitogens in inhibited by E type prostaglandins (PGE). It has been reported that the leukocytes of patients with multiple sclerosis (MS) are resistant to this inhibitory effect. We have studied 10 MS patients using an indirect technique for measuring LIF activity. This allows separation of the effects of PGE on lymphocyte LIF production from the effects on the action of LIF on the target granulocytes. There was no difference between normal subjects and MS patients in the effects of prostaglandins E1 and E2 in concentrations of 2.5 and 0.25 microgram per milliliter, on LIF production, or on granulocyte response to LIF. These results do not support the hypothesis that the chronic inflammatory process in MS is the result of resistance to the normal inhibitory effects of PGE on the cellular immune response.

Idiopathic steroid responsive chronic lymphocytic meningitis--clinical features and long-term outcome in 17 patients.

BACKGROUND: Some patients with chronic lymphocytic meningitis appear to respond to corticosteroid treatment, but investigations fail to identify the cause. The use of corticosteroids in patients with chronic meningitis is controversial and the long-term outcome is unclear. AIM: To review the long-term outcome in 17 patients with the syndrome of idiopathic steroid responsive chronic lymphocytic meningitis. METHODS: Review of patients' records and examination of surviving patients 1.5 to 17.5 years (median 8.8 years) after presentation. RESULTS: The cause of the steroid responsive chronic meningitis was found during follow up in two of the 17 patients: Wegener's granulomatosis and multiple sclerosis in one patient each. A cause was not found in the other 15 patients. Seven of these patients eventually recovered and corticosteroids were withdrawn after six weeks to six years without a recurrence of symptoms. Four patients improved transiently, but died six months to 26 years after starting treatment; in two, steroids were withdrawn a few weeks before death. Four patients had active chronic meningitis and reduction in the dose of corticosteroids was associated with a recurrence of symptoms. Leptomeningeal and brain biopsy in eight patients showed non-specific abnormalities which were not helpful in the diagnosis. CONCLUSIONS: A subgroup of patients with idiopathic chronic lymphocytic meningitis responds to corticosteroids. Leptomeningeal biopsy and long-term follow up seldom identify the underlying cause in these patients. Steroid treatment must be undertaken with caution, even after infective causes of chronic meningitis have been excluded.

A structured panel interview and group exercise in the selection of medical students.

The selection of entrants for medical school, as now widely agreed, should include measurements of non-academic as well as academic attributes. The authors have developed a process to assess the personal attributes of applicants. This included a structured panel interview carried out twice on each applicant by independent interviewers and a group exercise in which several applicants were observed whilst discussing a problem. Training for interviewers took the form of a half-day interactive workshop. One hundred and forty-one school-leaving applicants completed the new assessment. No relationship was found between academic achievement as reflected by marks in a national examination and scores in the panel interview, the group exercise, or the school principal's report. However, significant intercorrelations were found between the panel interview, group exercise and school report. The results of this experience have encouraged the Auckland School to continue to explore methods to measure these attributes in a carefully controlled study.

Brain biopsy in the management of focal encephalitis.

The value of a brain biopsy in diagnosis and management of suspected herpes simplex encephalitis was studied in 29 patients (16 prospectively and 13 retrospectively). The biopsy showed herpes simplex encephalitis in eight, culture-negative encephalitis in 14, and was normal in three patients. It provided an alternative diagnosis in four patients, for two of whom curative treatment was available. The biopsy was complicated by a fatal intracranial haemorrhage in one patient. The low yield of alternative diagnoses suggests that a brain biopsy is not justified in the routine investigation of focal encephalitis.

Serial magnetic resonance scanning in multiple sclerosis: a second prospective study in relapsing patients.

A prospective study of serial magnetic resonance (MR) scans of the brain was carried out every 2 weeks for 4 to 6 months in 9 patients with mild, clinically definite, relapsing/remitting multiple sclerosis (MS). Six of the 9 patients developed a total of 12 asymptomatic new lesions in various parts of the brain. In none of the patients were the changes on MR scan accompanied by relevant new neurological symptoms or signs. New MR lesions had a characteristic temporal profile, reaching a maximum size in approximately 4 weeks before gradually shrinking, usually leaving a small residual abnormality indistinguishable from chronic MS lesions. The frequent occurrence of new asymptomatic lesions indicates that MS may be a more active process even in mildly affected asymptomatic patients than has been previously realized. The results emphasize the potential importance of using MR scanning to measure disease activity in laboratory studies of MS and in the assessment of treatment, particularly in asymptomatic patients in the early stages. We suggest that the expanding and contracting new lesions are the basic or primary lesion in MS, that the characteristic demyelinated plaque is represented by the small residual area that these lesions shrink down to, and that the typical collection of scattered white matter lesions in chronic MS may represent the accumulated residua of dozens or more of these active lesions occurring over many years.