A case of cerebral gigantism with cerebellar atrophy

A 37-year-old housewife, who had physical characteristics of cerebral gigantism, such as the tall stature, acromegaly, macrocephalia, high arched palate and antimongoloid slant, developed cerebellar ataxia and dysarthria. Her mother, uncle and grandmother were also reported to have slowly progressive gait disturbance. Her mother was also tall. Endocrinological studies failed to show any definite abnormality. CT and MRI revealed remarkable cerebellar atrophy. Though cerebral gigantism is often associated with clumsiness and incoordination, the etiology of the ataxia is poorly understood. This case indicates that the ataxia in cerebral gigantism may be, at least partly, caused by cerebellar atrophy.     

Antitumor effect of a benzoylphenylurea derivative HO-221

HO-221, N-[4-(5-Bromo-2-pyrimidinyloxy)-3-chlorophenyl]-N'-(2-nitrobenzoyl ) urea is a novel benzoylphenylurea derivative. We had interested in various pharmacological actions of benzoylphenylurea compounds. Therefore, many compounds were synthetized and tested in various screening systems. In the process with these tests, we found HO-221 which showed an excellent antitumor activity. The antitumor activity of HO-221 was judged from the survival time and the tumor weight of experimented tumor-bearing animals. HO-221 preparation was orally administered. The compound exhibited significant effects against various animal tumors (P388, L1210, M5076, LLC, C38, S180, W256), and especially effective against the solid tumors. HO-221 was also markedly effective to MX-1 and LX-1 implanted into nude mice. However, the effect against mouse B16 melanoma was moderate. In addition, HO-221 showed a schedule dependency and once every 4 or 7 days treatments were most effective. The antitumor activities of the compound against advanced L1210 and Lewis lung tumors were examined. Tegafur and ara-C were used as reference drug for the study. Three agents showed the antitumor activities against L1210. Against Lewis lung carcinoma, HO-221 showed both the increase of life span and the tumor growth inhibition. On the other hand, tegafur and ara-C were ineffective for the increase of life span.     

Delayed finger opening caused by a midbrain lesion]

We report a 25 year-old woman with delayed finger opening caused by a midbrain lesion. Magnetic resonance imaging revealed an abnormal intensity area in the left midbrain in the vascular territory corresponding to the mesencephalic inferomedian branch and central posteromedian branch of posterior cerebral artery. Clinically, she suffered from Weber's syndrome, and in the course of recovery, she developed marked delayed finger opening. Her weakness was slight, this delayed finger opening was observed only after maximal grip, and she required about ten seconds to open her right fingers. She could open and grip her fingers easily and quickly in moderate contraction. In the electromyogram, the action potentials of flexor digitorum profundus muscle did not disappear promptly upon instruction to open her fingers, and they lasted for approximately six seconds with gradual decrease. On the other hand, the action potentials of extensor digitorum muscle were reciprocally inhibited and increased gradually after the decrease of the flexor muscle potentials. The abnormally continuing discharges in the flexor muscles were thought to be the cause of her delayed finger opening. The phenomenon of delayed finger opening caused by cervical root lesions, peripheral nerve lesions, or a brain stem (pons-medulla oblongata) lesion have been reported. But, the phenomenon caused by a midbrain lesion, like our case, has not been reported. The present case was different from the previously reported cases with this phenomenon showing simultaneous discharges from both agonistic and antagonistic muscles.     

A case of Hoffmann's syndrome with peripheral neuropathy and various pathological findings in muscle biopsy

A 58-year-old hypothyroid man developed numbness in the extremities and muscular hypertrophy without pain. Needle electromyography was normal. Nerve conduction study revealed severe entrapment and impaired conduction velocity. Muscle biopsy demonstrated small group atrophy, scattered hypertrophic fibers, interstitial fibrosis and subsarcolemmal vaculoes. These vacuoles were stained dark with NADH-TR. On electron microscopy these vacuoles were filled with aggregated mitochondria and glycogen particles. These myopathological findings have been rarely described in Hoffmann's syndrome or Kocher-Debré-Semelaigne syndrome in the literature. Sural nerve biopsy revealed a loss of myelinated fibers in large diameter and relative increase in small diameter fibers. Teased fiber examination demonstrated segmental demyelination. The nerve biopsy findings indicate slowly progressive axonopathy. In Hoffmann's syndrome, associated peripheral neuropathy may play some role in the manifestation of clinical signs and symptoms including muscle weakness and some sensory disturbance.     

Encephalopathy due to toluene sniffing. Report of a case with magnetic resonance imaging

A 27-year-old man with a 10-year history of toluene abuse developed dementia, cerebellar ataxia, dysarthria and pyramidal signs. Magnetic resonance imaging (MRI) revealed atrophy of the cerebrum, corpus callosum, cerebellum and brainstem. The internal capsule showed abnormal intensity. Chronic toluene abuse may affect not only the cerebral and cerebellar cortex or brainstem but also the subcortical cerebral white matter. MRI may be a sensitive tool to use in evaluating the severity and prognosis of the neurological syndrome resulting from toluene abuse.     

Morphometric study of cervical anterior horn cells and pyramidal tracts in medulla oblongata and the spinal cord in patients with cerebrovascular diseases

To clarify the effect of damage to the upper motor neurons on lower motor neurons, quantitative studies were made regarding the cross-sectional area and the number of the individual anterior horn cells in the lateral nuclear cell groups of the 5th segment of the cervical spinal cord (C5), and regarding the cross-sectional areas of the pyramidal tract in the medulla, and in the spinal cord at the C5 and L2 levels. The subjects included 45 patients with cerebrovascular disease (CVD), and 50 age-matched controls without neurological disease. The medullary pyramid (MP) and the ventral funiculus (VF), ipsilateral to the hemispheric lesion, were compared with the MP and VF of the other, unaffected, side. The ventro-lateral funiculus (VLF), anterior horn (AH) and C5 anterior horn cell (AHC), contralateral to the lesion, were also compared with the VLF, AH, and AHC of the other, unaffected, side. The AHC area (mean cross-sectional area of anterior horn cells) and the MP area, VF area, VLF area, AH area (mean cross-sectional areas of MP, VF, VLF and AH) associated with the hemispheric lesional side were significantly decreased, compared with those of the unaffected sides and the controls. However, there was no significant difference in AHC number between the affected and unaffected sides in patients with CVD, nor between the affected side and the controls. In order to examine the relationship between a decrease in AHC area and degree of paralysis, CVD patients were divided into two groups according to degree of muscle strength: the severe and mild paralysis groups.(ABSTRACT TRUNCATED AT 250 WORDS)     

Ferruginous bodies in the lungs of the general population during a 45-year period and mineralogical examination

In order to determine the level of asbestos pollution in the lungs of members of the general population in and around Tokyo, the incidence of ferruginous bodies in autopsied or resected lungs during 5 periods over the 45 years from 1937 to 1981 was studied by a light microscopy. Core fibres, after removal of their ferruginous coatings with oxalic acid, and uncoated fibres were analyzed using a scanning electron microscope equipped with a Kevex energy-dispersive X-ray spectrometer. The incidence of ferruginous bodies in 5 g (wet) of digested lung tissue was shown to be 10% in period I (1937-1941), 18% in period II (1947-1951), 70% in period III (1958-1963), 74.4% in period IV (1970-1973) and 81.0% in period V (1980-1981). The major types of core fibres of ferruginous bodies were found to be asbestos, including amosite, crocidolite, chrysotile and the tremolite-actiolite series, but a small number of fibers of materials other than asbestos were also detected. In contrast, a large number of short fibers less than 5 microns in length in 1 g of wet lung tissue were classified as belonging to Mg + Si fibers (the ratio of Mg to Si components being 30% or over) and presumed to be chrysotile. Thus an annual increase in asbestos deposition in lungs of people living in and around Tokyo has been demonstrated and fine chrysotile fibers less than 5 microns in length seem to be the main type of deposited fibers.