Comparative studies on clinical features and CSF proteins of motor neuron disease patients.

Clinical features such as types of diseases, sex ratio, age of onset, sites of initial involvement, the appearance of bulbar signs, and duration of illness were studied in 52 patients with motor neuron disease (MND) with a special reference to immunoglobulin in cerebrospinal fluid (CSF) and serum. Although MND has been thought to be a degenerative disease of unknown cause, our data suggested there are some immunological abnormalities in this disease. The duration of illness and the abnormalities of CSF immunoglobulins appeared to be correlated with the type of disease and the site of initial involvement. However, whether or not these abnormalities in CSF immunoglobulins are directly related to the pathogenesis of MND remains unclear.

Linoleate and fatty acid compositions in the serum lipids of Japanese patients with multiple sclerosis.

Serum fatty acid compositions were determined in 21 Japanese patients with multiple sclerosis and 14 neurological controls. No statistical difference was found either for linoleic acid or for arachidonic acid between the 2 groups. It may be that neither serum linoleic acid nor arachidonic acid is inevitably associated with the pathogenesis of multiple sclerosis.

Increased frequency of lymphocytotoxic antibodies in patients with multiple sclerosis correlated with disease activity.

Cold-reactive lymphocytotoxic antibodies (LCA) were investigated in sera from patients with multiple sclerosis (MS) in relation to disease activity. When a serum with more than 20% cytotoxicity is considered positive for the presence of LCA, the positive frequency at remission was 21% (6/28), but at exacerbation it was 83% (10/12). All the sera from normal controls were negative. The average of cytotoxicity expressed as a % killing of target cells by sera at remission was 13%; on the other hand, at exacerbation it was 39%. These results showed that in multiple sclerosis the level of LCA was not persistently high, but increased correlating with the disease activity. From these findings and previous experiments of rescue of measles virus antigens, it was suggested that multiple sclerosis is likely a virus-induced autoimmune disease.

Absence of Carp's agent in sera of patients with multiple sclerosis in Japan.

A study was devised to test for Carp's agent in the sera of Japanese patients with multiple sclerosis (MS). The sera were obtained from 17 patients with definite MS and six patients with possible MS. Strain C3H mice were given an intraperitoneal injection of 0.1 mL of each serum. One week before and 1, 3, and 6 weeks after inoculation, blood drops from tail tip were smeared and polymorphonuclear leukocytes (PMNLs) were counted. Although the experimental animals were kept under careful control, substantial fluctuation in the number of PMNLs occurred, and the variations of PMNL count in the experimental groups were all within the normal range. We conclude that there is no Carp's agent in the sera of patients with MS in Japan.

Delineation of voluntary movement in Parkinson's disease with the manual control system.

An assessment of the dynamic characteristics of voluntary movement in 20 patients with Parkinson's disease and 20 normal subjects was performed by the manual control system. The following eight parameters were estimated and mapped on the plane with eight axes: gain constant between the input and the output, cut frequency for evaluation of the frequency response of human operator, dead time, square error, correlation coefficient, and three different points of coherency for the measurement of the linearity of human operation. In Parkinson's disease, we found a low gain constant of 8.34 +/- 9.64 dB (mean +/- S.D.) (26.5 +/- 2.12 in controls), a narrow cut frequency of 1.08 +/- 0.91 radians/sec (5.65 +2- 1.25), a long dead time of 0.79 +/- 0.27 sec (0.19 +/- 0.05), a large square error, a low correlation coefficient, and low points of coherency. Among the eight parameters, the gain constant and the dead time proved to be the most useful for quantitative assessment of motor disability for this disorder. Our method promises to be valuable to confirm the effects of drug therapy and the progression of the disease.

Cytological and immunological examination of cerebrospinal fluid in 9 patients with Ramsay Hunt's syndrome.

The purpose of the present study was to carry out the immunological and cytological examination of cerebrospinal fluid in 9 patients with Ramsay Hunt's syndrome. The results obtained were as follows: Among the serum immunoglobulins, IgG in 1 and IgM in 5 of 9 cases increased. The Varicella Zoster antibody titer was significantly elevated in sera of all 9 cases and in CSF of all 7 examined cases. There ws positive fluorescence to Varicella Zoster virus in 2 (Cases 8 and 9) of 4 cases in which CSF smears were examined. The cells showing fluorescence were 10.5% in the former and 1.5% in the latter. Pleocytosis was found in all cases and CSF cell counts ranged from 52 to 2,000/3 mm3 in the early stage. In 3 cases immunoglobulin containing cells (IgG and IgM) were seen about 2-20% within 2 weeks of onset. IgG contents in all and IgG% in 8 of 9 cases, IgA concentration in 7 and IgA% in 7 of 9 cases were increased. These results support the view that the Varicella Zoster virus spreads to the meninges and CSF from ganglions in patients with Ramsay Hunt's syndromes.

Cytological examination of cerebrospinal fluid in eight patients with neuro-Behçet's disease.

In eight patients with Neuro-Behçet's disease cerebrospinal fluid (CSF) cells were studied in relation to the clinical symptoms, clinical course and treatment. The results obtained were as follows: (1) The mean value of CSF cell counts was 126 +/- 177/3 mm3 in 88 specimens from the patients, and 2.9 +/- 2.4/3 mm3 in 30 specimens from healthy control subjects, the former being significantly higher than the latter (p less than 0.01). (2) In the patients' CSF, the average percentages of mononuclear and polymorphonuclear cells were 67 +/- 23% and 32 +/- 23%, respectively. (3) In CSF cytogram from 37 specimens from the patients, the average percentage of small lymphocytes was 59 +/- 19%, which was significantly lower (p less than 0.01) than control (71 +/- 8%). The average percentage of activated lymphocytes, activated monocytes and neutrophils which were not observed in control were 0.5 +/- 1.2%. 1.9 +/- 2.2% and 17 +/- 16%, respectively. (4) The ratio of nucleic acids (RNA/DNA) measured by microspectrophotometry (Zeiss UMSP-1) in CSF lymphocytes stained with methylgreen and pyronin was 0.58 +/- 0.12 in 17 specimens from the patients and 0.55 +/- 0.07% in 10 specimens from the control; there was no significant difference between them. These results suggested that the pleocytosis in Neuro-Behçet's disease may represent perivascular inflammatory process in the central nervous system.

Acute disseminated encephalomyelitis after influenza vaccination.

A 12-year-old, previously healthy boy was seen with typical signs of acute disseminated encephalomyelitis four days after influenza vaccination. Computerized tomography demonstrated an extensive subcortical low-density area in the left cerebral hemisphere. The CSF contained elevated levels of immunoglobulins, especially IgG, and an abnormal kappa:lambda chain ratio. Skin tests for hypersensitivity to egg protein were negative, but reaugmentation of CSF protein and immunoglobulin levels was observed within ten days after skin tests.

Changes in free amino acids of cerebrospinal fluid and plasma in various neurological diseases.

By means of liquid chromatography we investigated free amino acids in cerebrospinal fluid (CSF) (161 subjects) and in plasma (138 subjects) in various neurological diseases and in controls. A general increase in CSF amino acids was found in Group C (samples exhibiting both pleocytosis and increasing CSF protein concentration) and in "spinal canal block" and "neuro-Behçet's syndrome". Also, some abnormalities were detected in these groups with regard to changes in CSF cells, protein or IgG%. In the case of "Guillain-Barré syndrome" we noted an increase in CSF alanine, glycine, methionine, arginine and the branched-chain group. In "multiple sclerosis" an increase was evident in CSF alanine, in plasma alanine and arginine, and in the plasma: CSF ratio of tyrosine. In patients of "motor neuron diseases (MND)" CSF glutamic acid, alanine, histidine and arginine all indicated an increase, along with the plasma: CSF ratio of tyrosine. In Group C and in "neuro-Behçet's syndrome" glycine, methionine and lysine were lowered in the plasma: CSF ratio. Also, the ratio of valine in the former and histidine in the latter indicated a decrease. These results can be supposed to reflect either biochemical abnormalities of plasma and/or the CNS, or changes in the transport systems of the blood-brain barrier.