A COMPARISON BETWEEN AGAR GEL ELECTROPHORESIS and CSF SERUM QUOTIENTS of IgG and ALBUMIN IN NEUROLOGICAL DISEASES

CSF and serum was obtained from 216 patients with neurological or psychoneurotic symptoms and the concentrations of albumin and IgG were immunologically determined. The IgG/albumin index, calculated as the quotient of the CSF/serum ratios of IgG and albumin was compared with electrophoresis on agar gel. In "normal" cases, the IgG/albumin index was between 0.26-0.66. Pathological electrophoresis, i.e. with two or more IgG bands in the gamma globulin region was found in 85 per cent of the MS patients; in 29 per cent of the patients with a possible demyelinating disease; in 41 per cent of patients with CNS infection; and in 4 per cent of patients with other neurological disorders; whereas an increased IgG/albumin index ( greater than 0.66) was found in 88 per cent of the MS patients; in 43 per cent of the patients with a possible demyelinating disease; in 50 per cent of the patients with CNS infection; in 11 per cent of patients with immunological disorders; and in 18 per cent of patients with other neurological diseases. The increase of the IgG/albumin index was sometimes moderate (0.67-0.90), except in patients with MS, syphilis and other CNS infections, where a pathological electrophoresis combined with an IgG/albumin index above 1.0 was found to be a valuable support for the clinical diagnosis.     

Clinical and cerebrospinal fluid findings in lymphocytic meningo-radiculitis (Bannwarth's syndrome)

11 patients with lymphocytic meningo-radiculitis (Bannwarths syndrome) are described. The disease is characterized by intensive pain, often radiating and migrating, and neurological deficits, i.e. peripheral facial palsies, as well as disseminated radiculopathies. In 4 patients the onset was preceeded by an arthropode bite, close to which 3 patients exhibited an erythema chronicum migrans.
In the cerebrospinal fluid there was a mononuclear pleocytosis, predominately by lymphocytes. In the early stages, a plasma cell reaction was frequently encountered. Protein analysis indicated a defect in the blood brain barrier and an intrathecal synthesis of IgG.
All patients recovered almost completely from their neurological symptoms within 1–2 months, irrespective of treatment.     

Comparison between induced platelet aggregation and circulating platelet aggregates as platelet function tests in patients with transient ischemic attacks

Routine blood analysis, platelet counts, number of circulating platelet aggregates (CPA) and platelet aggregation in vitro against adenosine-diphosphate (ADP), epinephrine and collagene were studied in 45 healthy controls, in 10 hospitalized patients with other neurological diseases than stroke and in 12 patients with transient ischemic attacks (TIA) before and after prophylactive treatment with anticoagulants (AC) or anti-platelet drugs (APD).
Except for lower hemoglobin and hematocrit levels in women, sex, smoking, oral contraceptives or pregnancy did not significantly influence the routine blood parameter. Smoking females taking oral contraceptives had an increased number of CPA and the most easily induced aggregation in vitro .
Patients with TIA had no significant differences in blood or platelet findings versus the healthy controls (except smoking females on oral contraceptives) or the non-stroke patients, even though individual patients could have high numbers of CPA and an easily induced platelet aggregation in vitro . Treatment with AC did not influence platelet function, whereas APD therapy decreased the number of CPA and inhibited the secondary platelet aggregation in vitro .     

Neuronal intranuclear inclusion disease: report on a case originally diagnosed as dopa-responsive dystonia with Lewy bodies.

Neuronal intranuclear inclusion disease (NIID) is a rare neurodegenerative disorder with a heterogeneous clinical picture characterized by the presence of eosinophilic intranuclear inclusions in neuronal and glial cells. We describe a case, reported 12 years ago as dopa-responsive dystonia (DRD) with Lewy body pathology. Pathological re-examination has led to a revised diagnosis of neuronal intranuclear inclusion disease. This rare condition, which may be diagnosed in life with a full thickness rectal biopsy, needs to be considered in the differential diagnosis of any case presenting as progressive juvenile parkinsonism (JP) or dystonia.     

Soluble brain proteins in dialysis encephalopathy

Summary Soluble brain proteins were analyzed regionally in a case of dialysis encephalopathy and compared to the findings in patients dying from uncomplicated uremia and acute myocardial infarction. Sixteen individual proteins were quantitated and the results may indicate that dialysis encephalopathy is accompanied by defects in the blood-brain barrier. However, such a postulate does not explain why certain proteins (-trace protein) occur at very low concentrations in dialysis encephalopathy.     

DIAGNOSTIC VALUE OF VISUAL EVOKED RESPONSE, CLINICAL EYE EXAMINATION AND CSF ANALYSIS IN CHRONIC MYELOPATHY

The results of neuro-ophthalmological examination, visual evoked responses (VER) to pattern reversal stimulation and cerebrospinal fluid (CSF) analysis were compared in 25 patients with myelopathy of unknown etiology and without subjective symptoms of involvement of CNS outside the spinal cord. Delayed latencies of VER indicating a disseminated disease were found in 76 per cent of the patients. In 64 per cent of the patients, CSF showed abnormalities similar to those found in MS. Pathological findings at the clinical eye examination consistent with such an etiology was found in 36 per cent of the cases. It is suggested that a large proportion of patients with myelopathy of unknown etiology suffer from a disseminated demyelinating disorder similar to MS. By a combination of neuro-ophthalmological, VER and CSF examinations such an etiology can be established with reasonable certainty, and more troublesome investigations such as myelographies and spinal angiographies may be restricted to patients in whom the etiology still remains unclear.     

Tolcapone added to levodopa in stable parkinsonian patients: A double-blind placebo-controlled study

The primary objective of this study was to assess the effect of tolcapone on levodopa dosage in parkinsonian patients whose “wearing-off” phenomenon has been controlled with more frequent levodopa dosage. After a 1-week placebo run-in, 97 patients were assigned randomly to receive placebo or tolcapone 200 or 400 mg three times daily (t.i.d.). Levodopa dosage was reduced by −35% on day 1 of study and subsequently retitrated as required. After 6 weeks, the tolcapone groups crossed over to receive the other dose for a further 3 weeks for exploratory purposes. Both tolcapone groups had greater reductions in levodopa dosage than the placebo group at week 6 (not statistically different). The 200-mg t.i.d. group showed greatest improvement in estimated mean scores for all efficacy parameters (p < 0.05 versus placebo for change in Unified Parkinson's Disease Rating Scale Subscale II). Fewer dopaminergic and nondopaminergic adverse events were associated with tolcapone 200 mg t.i.d. than with tolcapone 400 mg t.i.d. The most frequently reported dopaminergic adverse events were nausea, cramps, dyskinesia, and dystonia. The most frequently reported unanticipated adverse event was diarrhea. Tolcapone 200 mg t.i.d. may provide additional benefit to patients with moderately advanced Parkinson's disease with treated “wearing-off” phenomenon.     

HLA—Determination in Families with Hereditary Ataxia

In three families with hereditary ataxia, where the inheritance pattern was autosomal and dominant, HLA antigens were determined in 25 members. In two of the families, HLA linkage of disease was suggested, whereas in the third family, the data did not directly support this concept, since two recombinational events between the postulated locus for disease and the HLA region had to be assumed. However, with this assumption, our data are compatible with those of one family described recently (Jackson et al. 1977) implying the presence on the sixth chromosome, outside the HLA region, of a locus that determines the development of spino cerebellar ataxia (SCA). Further tests with definition of enzyme markers will have to be performed before conclusions as to HLA linkage of a postulated SCA gene can be made.     

Comparison of Antibodies Against Different Viruses in Cerebrospinal Fluid and Serum Samples from Patients with Multiple Sclerosis

The occurrence of a local production in the central nervous system (CNS) of antibodies against different selected viruses was analyzed by comparison of titers in serum and cerebrospinal fluid samples from groups of 50 patients with multiple sclerosis from Finland, Norway, and Sweden. Measles antibodies were determined in hemagglutination inhibition, hemolysis inhibition, and nucleocapsid complement fixation tests; mumps, parainfluenza virus type 1, and rubella virus antibodies were determined in hemagglutination inhibition tests; and herpes simplex virus type 1 antibodies were determined in passive hemagglutination tests. For reference purposes tests were also made for adenovirus antibodies in penton hemagglutination enhancement tests and poliovirus antibodies in neutralization enhancement tests. Among the 150 multiple sclerosis patients, a local production of antibodies against measles virus was found in the CNS in 57%, against rubella virus in 19%, mumps virus in 15%, herpes simplex virus type 1 in 11%, and parainfluenza virus type 1 (Sendai) in 3%. A local production in the CNS of antibodies against any of the viruses studied was found in 71% of multiple sclerosis patients. These included 48, 16, and 7% that produced antibodies to one, two, and three or more viruses, respectively.     

Diagnostic value of determinations of lysosomal hydrolases in CSF of patients with neurological diseases

The activities of four lysosomal acid hydrolases, beta-galactosidase, alpha-mannosidase at pH 4.5 and 5.5, N-acetyl-beta-glucosaminidase, and acid phosphatase, have been measured in serum and cerebrospinal fluid from 179 patients with different neurological diseases and from 20 healthy controls. In patients with tumours, decreased activity of beta-galactosidase was found in both serum and cerebrospinal fluid, and in patients with multiple sclerosis and collagen diseases, decreased activities of beta-galactosidase and N-acetyl-beta-glucosaminidase were found in cerebrospinal fluid. The variations of enzyme activities were great between the individual patients even with these groups and analysis of lysosomal enzymes seems to have a very poor clinical value.