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To investigate the usefulness of expectorated sputum in the assessment of airway inflammation by smoking. I examined cytokine expression in the sputum of healthy smokers and non-smokers, and compared them before, and at 8,12 and 16 weeks after, cessation of smoking. Four cytokines, interleukin (IL) 8, interferon (IFN)-gamma, tumor necrosis factor (TNF)-alpha, and IL-10, were assessed by immunohistochemistry. In smokers, the percentage of bronchial epithelial (BE) cells and macrophages with high intracellular contents of the pro-inflammatory cytokines IL-8, IFN-gamma and TNF-alpha, were significantly increased in comparison with non-smokers. In contrast, expression of IL-10 was significantly decreased in smokers. After smoking cessation, expression of IL-8 was significantly decreased by the 16th week, that of IFN-gamma was significantly decreased by the 12th to the 16th week, but that of TNF-alpha was unchanged even at 16 weeks post-cessation, and that of IL-10 was significantly increased at that time. Cytokine expression in BE cells was similar to that in macrophages. From the data, we concluded that expectorated sputum is useful for assessing airway inflammation due to smoking and for following the series of changes that follow smoking cessation.

To explore the optimum dose of intravenous immunoglobulin (i.v.Ig) for treating patients with chronic inflammatory demyelinating polyrneuropathy and multifocal motor neuropathy, we compared the usefulness of i.v.Ig among 3 treatment doses. Fifty-nine patients were randomly divided into three treatment dosage groups: 20 patients for Group I using 50 mg/kg/day x 5 days, 19 patients Group II using 200 mg/kg/day x 5 days, and 20 patients Group III using 400 mg/kg/day x 5 days. We assessed clinically and electrophysiologically the effectiveness of the treatment at 5 weeks after the initial infusion. For patients in Group I and II who had not improved (or worsened) with the first treatment, we gave a one-step larger dose in the second treatment (i.e. 200 mg/kg/day x 5 days for those who had been given 50 mg/kg/day x 5 days, 400 mg/kg/day x 5 days for those who had been given 200 mg/kg/day x 5 days) after more than 9 weeks. We found that 15% of the patients in Group I, 21% in Group II and 60% in Group III improved dose-dependently with the first intravenous immunoglobulin treatment. Seven (47%) of 16 patients in Group I and 4 (40%) of 11 patients in Group II improved after the second treatment with larger doses. Adverse reactions including chill sensation, fever, skin eruption and increase in blood GOT and GPT levels were transient and mild. One patient in Group III developed left hemiparesis showing the small infarction in the right thalamus during the course of the treatment, but the symptom was mild. In conclusion, the high-dose intravenous immunoglobulin therapy (400 mg/kg/day x 5 days) is useful for treating patients with CIDP and MMN, although care must be taken of the risk of causing cerebral infarctions.

We report a 55-year-old woman who developed symptoms resembling parkinsonism. Her psychiatric symptoms in the early stage, cervical dystonia and tremor increasing on movement were consistent with manganese poisoning. Manganese levels were elevated to 1.5 micrograms/l in the serum (normal; 0.3-1.1 micrograms/l) and to 1.4 micrograms/l in the urine (normal; less than 1.2 micrograms/l). Intravenous infusion of calcium disodium editate (CaEDTA; chelating agent) was followed by the marked excretion of manganese (27.3 micrograms/l) in the urine. These findings support manganese poisoning. Manganese poisoning is a disease which results from chronic exposure to manganese, but the source of manganese exposure remained obscure in this patient. T1-weighted MRI of the brain showed symmetric high signal intensity in the globus pallidus without any abnormality on T2-weighted images. There is a report that manganese induced brain lesions in Macaca fascicularis as revealed by MRI and the fascicularis developed signs of unsteady gait and hypoactivity. The patient responded to treatment with CaEDTA and the second MRI demonstrated regression of abnormal signal intensity. This may be due to enhanced manganese excretion. To our knowledge, this is the first case of probable manganese-induced human parkinsonism whom changes in MRI were noted after treatment with CaEDTA.

A 52-year-old right-handed man presented progressive dystonia and apraxia of his right hand of five years' duration. He also suffered from parkinsonian features such as rigidity or impaired postural reflexes. Serial investigation of brain MRI revealed progressive cerebral atrophy, which started in the left parietal lobe, and subsequently extended to both hemispheres. He was clinically diagnosed as corticobasal degeneration. He could not point at any part of his own body in response to verbal or visual commands. On the other hand, he could point at every part of the examiner's body or of the illustrated body image. Deep sensations and linguistic functions were not involved. This cognitive impairment was regarded as autotopagnosia. In contrast with inability to recognize any part of the own body in response to the commands, he could name every part of his body as soon as the examiner touched there. Moreover, his symptoms of autotopagnosia were ameliorated by looking at himself in a mirror; he could point at any part of his own body. Disconnection between primary proprioceptive sensory area and the center of body schema was thought to be the mechanism of autotopagnosia in this patient, because the impairment improved with the aid of visual or tactile informations. We speculated the lesion was the left parietal lobe.

Lower lung field tuberculosis has been considered as rare disease, but it was observed about ten percent in the literature. It seemed that the important problems in lower lung field tuberculosis are delay and difficulty of diagnosis. The materials for this report was accumulated over a period of 10 years, from January 1983 to December 1992 at the National Sanatorium Fukuoka-Higashi Hospital. The criteria for lower lung field tuberculosis are: (1) disease confined to lower lobe except for S6a, middle lobe and lingular segment, (2) bacteriologically and/or histologically proved diagnosis of tuberculosis, (3) clinically considered as tuberculosis for which improved by only anti-tuberculous drugs. During this period, 672 new patients suffering from active pulmonary tuberculosis were admitted to the sanatorium. Of the total of 672 patients, 57 patients were selected as the criteria for lower lung field tuberculosis. The incidence of them was 8.5 percent of the total new admissions. Of these 57 patients, 30 were females and 27 males. The mean age of these 57 patients was 59.4 years ranging between 19 and 86 years. The incidence of them obtained through ten years did not show any tendency to increase or decrease. The respiratory and general symtoms most commonly observed were cough and chest pain. The average duration of used for diagnosis of the lower lung field tuberculosis over 65 years old was 71.7 days and it seemed to show the delay and difficulty of diagnosis.

Recent progress in neurology has revealed that hereditary olivo-ponto-cerebellar atrophy (OPCA) is in fact three different diseases. These are Menzel's disease, in which degeneration in the olivopontocerebellar (OPC) system is quite severe and similar to that of the patient described by Menzel in 1891, spinocerebellar ataxia 1 (SCA 1), in which the gene locus exists in the short arm of chromosome 6, and hereditary OPCA with retinal degeneration. We present a family with Menzel's disease, some of whom showed dementia and various extrapyramidal symptoms including tremor, myoclonus, and choreoathetoid involuntary movement.This dominant hereditary cerebellar ataxia family had five affected members in four generations. Neuropathological examination of one member (Case 3) revealed Menzel's disease. There was severe degeneration in the OPC system, the substantia nigra, Clarke's column, the posterior column, and the anterior horn of the spinal cord, and slight-to-moderate degeneration in the globus pallidus and subthalamic nucleus. However, the dentate nucleus, spinocerebellar tracts, and oculomotor nucleus including the medial longitudinal fasciculus were spared. The brain weight was 990 g. Case 1 (Case 3's grandmother) developed slowly progressive ataxia at the age of 55. She showed no involuntary movement or dementia. She died at 63 years of age. Case 2 (Case 3's mother) developed ataxia at 42 years of age, followed by tremor of the hands and head, and died at age 57. She did not show dementia. Case 3 (the autopsied case) developed progressive ataxia at 27 years of age, followed by mental deterioration, tremor, myoclonus, and generalized amyotrophy and sensory disturbance during her fifth decade.(ABSTRACT TRUNCATED AT 250 WORDS)

A 54-year-old woman was hospitalized because of paraparesis, sensory disturbance of the lower extremities, and dysuria. Laboratory data included an erythrocyte sedimentation rate (ESR) of 16 mm/h and lactate dehydrogenase (LDH) level of 459 IU/l. Myelogram showed an enlarged spinal cord at the L1 level, and spinal MRI revealed a high signal area on T2-weighted images. Spinal cord biopsy by thoraco-lumbar laminectomy showed thickening of the pia mater and neovascularization, but no malignant cells. Immediately after the operation, the patient displayed progressive mental deterioration. Cranial MRI showed widespread high signal areas in the cerebral white matter on T2-weighted images which resembled those of leukoencephalopathy. Steroid therapy was tried but was ineffective. Ten months after the onset of symptoms, a brief period of regression of both clinical symptoms and MRI appearances occurred following the administration of Inosiplex, subsequent progressive deterioration led to death 14 months after the onset of symptoms. LDH, C-reactive protein (CRP), and cerebrospinal fluid (CSF) protein were all elevated during the deterioration of her general status. Postmortem examination revealed a large tumor mass of the left adrenal gland which extended to the Th12, L1, and L2 vertebrae and remarkable brain edema. Microscopic mononuclear tumor cells were widespread but confined to the lumens of small vessels of the brain, spinal cord, spinal root and right adrenal gland, almost as if they were occluded. Cerebral white matter presented widespread multiple small infarcts and extensive myelin thinning. In addition to the left adrenal gland, interstitial invasions of tumor cells were discovered in the kidneys, iliopsoas muscles, oviducts, myocardium, lymph nodes, and thyroid gland.(ABSTRACT TRUNCATED AT 250 WORDS)

This report is to investigate neuropathological changes of the thalamus observed in progressive supranuclear palsy. Six autopsy cases were examined. They were 5 males and a female, whose age was between 57 and 72 years old. They developed dementia in various degrees and three of the cases showed periodic stuporous state and akinetic mutism in the last stage. The paraffined thin-sections were stained with hematoxylin-eosin, luxol-fast-blue, Holzer, modified Bielschowsky, Gallyas and anti-tau stainings. The histological changes were evaluated in the aspects of neuronal loss, fibrillary gliosis and neurofibrillary tangles. In conclusion, there were three types in six cases. The first was seen in two cases and that there appeared many Alzheimer's neurofibrillary tangles (NFTs) in medial nuclei, lateral and ventral nuclei as well as zona incerta and reticular nucleus. The second was observed in two cases and that the loss of nerve cells was severe, especially in medial nuclei and the dorsal part of lateral and ventral nuclei and fibrillary gliosis was observed beyond the lesions of neuronal loss. The third was that the appearance of NFTs was moderate in number and neuronal loss was minimal. Moreover, neuronal loss and NFTs of zona incerta were common in these cases. Argentophilic fibrillary inclusions were observed in the neuropil and astrocytes in all cases. It is important to investigate the correlations between neuropathological changes and clinical manifestations, especially in the peculiar type of dementia observed in PSP. The thalamic changes of PSP may play an important role in causing stuporous state and akinetic mutism as well as subcortical dementia.

A 48-year-old housewife was hospitalized because of spastic paraparesis accompanied by dysesthesia and amyotrophy in her lower extremities, and by urinary disturbances. At the age of 35 years she noticed tingling sensations in her right foot and gradually her gait became spastic. In a few years lumbago and the spinal form of intermittent claudication developed. When she was 41 years old muscle wasting was noticed in her right leg, and in four years the same feature was also seen in her lower left limb. Plain X-ray films showed a wide cervical canal with no signs of basilar impression. On MRI we could see a polycyst-like syrinx that extended over the entire spinal cord. In addition the surfaces of the lower thoracic cord seemed irregular, but arteriovenous malformation itself was not visualized. Neither hydrocephalus nor Chiari malformation was accompanied. Needle EMG examination revealed some neurogenic changes in the muscles of both lower limbs. Cystometry proved uninhibited contractions of the detrusor muscle and the existence of detrusor-sphincter dyssynergia. Myelography showed an enlarged spinal cord above the D11-12 level with a shadow of worm-like appearance between the D7-12 level. CSF protein was elevated. Spinal angiography confirmed the presence of single coiled vessels with a nidus at D8-10 that were fed by bilateral D10 intercostal arteries and drained to D7, D9 or D12. Embolization of these abnormal vessels was conducted, and since then the patient's condition has remained stable. Vascular malformation is a rare cause of secondary syringomyelia. Clinical manifestations are determined mainly by the level of vascular malformation and are not reflective of the level of syrinx.(ABSTRACT TRUNCATED AT 250 WORDS)

Acupuncture has been practiced in the treatment of many diseases in Japan. "Okibari" is one of the procedures in acupuncture treatment: a fine stainless steel or silver needle is inserted into the subcutaneous tissue through the skin, to remain in the subcutaneous tissue. A 57-year-old pharmacist was knocked down by a motorcycle in 1971, since then moderate weakness of left extremities and stiffness of muscles have remained as sequelae. She was consequently treated with acupuncture. Many small needles were inserted permanently in the nuchal, occipital and other areas of the body ten to twelve years before she developed gradual clumsiness and dysesthesia in her right hand in 1984. When she was admitted for the first time in 1985, neurological examination revealed left Horner's syndrome and diminished deep sensation in her right extremities with pseudo-athetosis of her right hand, along with spastic paresis of left extremities and right carpal tunnel syndrome. An old needle which had strayed into left dorsal medulla was considered to be responsible for these symptoms. In 1988 loss of pain and temperature sensation in the right side of her body below the shoulder, and diminished deep sensation of left extremities were appended, and weakness of her left extremities became aggravated. Pseudo-athetosis of her right hand was seen less prominently. In plain X-ray films many needle shadows were visualized. On CT scan needle shadows could be seen also in the left dorsal medulla, right cerebellum and in the subarachnoid space of left dorsal C1-C2 level.(ABSTRACT TRUNCATED AT 250 WORDS)

Idiopathic spasmodic torticollis is a type of focal dystonia. Major muscles which rotate the neck are M. sternocleidomastoideus (SCM) and M. splenius capitis (Spl) on both sides. In torticollis patients, its clinical characteristics could be understood as a vectorial summing up of tonus in bilateral SCM and Spl at rest. There is not any curative treatment for dystonia yet. A variety of medications and many types of surgical interventions have been tried without consistent or satisfactory results. In recent days local injection of botulinum-A toxin has shown to be effective in weakening focal dystonias. We used pure ethanol for local injection. Fourteen patients aged between 20-77 years (mean 48.9) were treated by alcoholisation. Disease duration ranged from 5 months to 12 years (mean 4.9 years). All had torticollis alone or had segmental dystonia containing spasmodic torticollis. Patients were recorded electromyographically using surface electrodes to make sure which neck muscles were hypertonic, and were rated before and after treatment according to the stages (0; normal-5; most severe). On the bases of these recordings the two most active muscles were selected for injection. Into the motor point of these muscles 1 ml of 1% lidocaine, and then the same dose of 99% ethanol were injected. This procedure was repeated on the mean ten times (6-14 times) every other week. The number of times of injection was decided in each case. Using the paired Student t test, there was a significant (p less than 0.01) improvement of the stage for the patients after injection, with a mean of 3.7 before treatment and 2.3 after treatment.(ABSTRACT TRUNCATED AT 250 WORDS)

The CT values of adrenal tumors were evaluated in 36 patients to determine the frequency and significance of negative CT values. Twenty-seven patients had adrenocortical adenomas (13 had primary aldosteronism, 12 had Cushing syndrome, and two had nonhyperfunctioning adenoma), one had adrenocortical carcinoma, and eight had pheochromocytomas. The CT values in primary aldosteronism (mean, 1.8 +/- 9.9 H) were lower than those in Cushing syndrome (27.6 +/- 12.0 H) and pheochromocytoma (35.9 +/- 9.8 H). Six adrenocortical adenomas had homogeneous, low CT attenuation, with CT values ranging from -20 to -0.4 H. Four of these had primary aldosteronism, one had Cushing syndrome, and one had nonhyperfunctioning adenoma. Our results suggest that adrenal tumors with homogeneous, slightly negative CT attenuation are likely to be adrenocortical adenomas, mainly primary aldosteronism and nonhyperfunctioning adenomas. This finding may be especially useful in diagnosing nonhyperfunctioning adenoma.

Progressive supranuclear palsy (PSP) is characterized by symptoms of disturbance of eye movement, nuchal rigidity, parkinsonism and subcortical dementia. Its pathology reveals that Alzheimer's neurofibrillary tangles (NFTs) are situated especially in the nuclei of basal ganglia and brainstem. It has been pointed out that NFTs observed in PSP are composed of straight tubules, the width of which is about 15 nm. This report is to reappraise the ultrastructure of NFTs observed in three cases of PSP. They are case 1; 69-year-old male, case 2; 62-year-old female and case 3; 65-year-old male. The clinical features of three cases were characterized by above-mentioned symptoms. Many NFTs were observed in brainstem of case 1 and 3. NFTs seen in brainstem of case 2 were in a small amount. In case 1 and 2, there were many NFTs in cerebral cortex, especially in parahippocampal gyri and other limbic areas. We examined the portions of frontal cerebral cortex, parahippocampal cortex, the basal nucleus of Meynert, oculomotor nucleus, substantia nigra, pontine nuclei and locus caeruleus of case 1, substantia nigra, red nucleus and inferior olivary nucleus of case 2, and Ammon's horn, globus pallidum, subthalamic nucleus, substantia nigra, oculomotor nucleus, pontine nuclei, locus ceruleus and inferior olivary nucleus of case 3. Ultrastructurally, NFTs of frontal cortex of case 1 consisted of twisted tubules and those of hippocampal and parahippocampal cortices of case 1 consisted of straight and twisted tubules, being observed separately in neurons. The NFTs of brainstem of case 1 and 2 were mainly composed of 12-17 nm straight tubules, and twisted tubules occasionally intermingled with straight components. In locus ceruleus of case 1, a single straight tubule could be seen in the bundles of twisted tubules. NFTs of case 3 were composed of only straight tubules, the width of which was about 15 nm. Twisted components were not observed in neurons of each nuclei of case 3. The characteristic ultrastructure of NFTs in PSP has been an appearance of straight tubules, however, twisted tubules were occasionally observed in this study. There are several case reports which demonstrated an appearance of both straight and twisted components. It is easily presumed that the process of aging takes part in the accumulation of twisted fibrils. However, it is now under consideration why twisted tubules are also observed in PSP. To summarize this study, the cases with many NFTs observed in widespread cerebral cortex may have a tendency to show both straight and twisted tubules.