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A 40-year-old Japanese man with acute myeloid leukemia received allogeneic bone marrow transplantation. On day 101, varicella-zoster virus (VZV) infection occurred, but was improved by administration of acyclovir and immunoglobulin. On day 119, he complained of numbness and double vision, and he was admitted due to exacerbation of the symptoms. The findings of cerebrospinal fluid and magnetic resonance image examination were consistent with the diagnosis of immune-mediated encephalomyelitis (IMEM). Intravenous immunoglobulin therapy was effective and his neurological findings dramatically improved without recurrence. IMEM is a rare non-infectious inflammatory demyelinating disease that can occur after transplantation. We herein describe a case report with a review of the associated literature.

We report a 57 year-old woman with polymyositis and interstitial pneumonia. With the steroid therapy alone, the decline of creatine kinase was insufficient and muscle strength was not improved. After the addition of tacrolimus 3 mg, serum creatine kinase declined, muscle strength was improved, and the dose of steroid could be tapered very smoothly. There was no remarkable change in the status of the interstitial pneumonia on the chest CT, but the vital capacity was improved. The improvement of the respiratory muscle strength might reduce the respiratory symptom and increase the daily activity in this case. It was possible that dose of steroid was tapered early with tacrolimus, and the side effect which accompanies long-term medication of steroids could be avoided. In polymyositis, tacrolimus is very useful medicine in the steroid-resistant case or in the case suffering from the side effect of steroids. There is possibility that tacrolimus becomes one of the choices of the treatment of polymyositis.

We report a 73-year-old man who suffered from an acute onset of dysphagia, cough, hoarseness and left facial and occipital pain. On the 44 days of illness, he was admitted to our clinic. A neurological examination revealed left IX, X and XI cranial nerve palsy. The diagnosis of Vernet's syndrome due to varicella-zoster virus (VZV) infection was made, based on the high titers of VZV antibody in serum. Magnetic resonance imaging revealed a unique nodular lesion with gadolinium enhancement at the medial side of the left jugular foramen. Clinical symptoms improved with intravenous high dose pulse methylprednisolone therapy. The clinical course suggests that the inflammation extended from the left X cranial nerve ganglion.

A 59-year-old Japanese woman, diagnosed with mixed connective tissue disease (MCTD) and chronic myelogenous leukemia (CML), developed several characteristic features of Behçet's disease (BD) after receiving alpha-interferon (IFN-alpha). She displayed recurrent oral aphtha, genital ulceration, erythema nodosum at the bilateral arm, and low grade fever. The skin of the femur where the IFN-alpha was injected showed modular acneiform changes. Skin biopsy revealed perivascular infiltration. The symptoms compatible with BD improved after discontinuation of IFN-alpha and the introduction of prednisolone and azathioprine. This is the first description of BD induced by IFN-alpha.

A 49-year-old man who had been suffering from poorly controlled diabetes mellitus for 20 years was admitted to our hospital with the chief complaints of diplopia and right ptosis. The onset of his symptoms was sudden. On admission, he had right palpebral ptosis, upward and left lateral gaze paresis of the right eye. Hess screen chart examination revealed paresis of the right superior rectus, inferior oblique and medial rectus muscles. T2 weighted magnetic resonance imaging (MRI) revealed high intensity area in the medial part of the right midbrain tegmentum where the oculomotor nucleus located, and diffusion weighted MRI demonstrated high intensity area which corresponded to the oculomotor fascicles of the midbrain. The latter represented Wallerian degeneration of the fascicles. The diagnosis of small infarction of the right oculomotor nucleus presenting ipsilateral external ophthalmoplegia was made. The superior rectus muscle is innervated by the neurons in the contralateral oculomotor nucleus, and the other external ocular muscles innervated by the third cranial nerve are conducted by the neurons in the ipsilateral oculomotor nucleus; therefore, unilateral oculomotor nuclear lesion generally causes ipsilateral ophthalmoplegia plus contralateral superior rectus palsy. Our case presented here shows that there may be such a case with oculomotor nuclear disturbance which masquerades with oculomotor fascicular or infranuclear disturbance; therefore, it is important to include these lesions in differential diagnosis.

Magnetic resonance images in some cases of amyotrophic lateral sclerosis (ALS) revealed abnormal signals in both the paraventricular white matter and in the posterior limbs of the internal capsule. We examined T2- and diffusion-weighted MR images of these lesions in 18 cases of ALS. There were symmetrical high-signal areas in the posterior limbs of the internal capsule in all of the cases. The high-signal areas in the internal capsule corresponded to the pyramidal tracts in the anatomical atlas by Talairach. In 5 of the cases of ALS, T2- weighted MR images showed discrete paraventricular white matter lesions as well. The mean age of the ALS patients with paraventricular white matter lesions was higher than that of the ALS patients without such lesions. Proton densities calculated from the conventional MR images were higher in both the capsular and paraventricular lesions. The diffusion coefficients perpendicular to the pyramidal tract in the internal capsula lesions were within the normal range, where as the diffusion coefficients in the paraventricular lesions were increased in all directions. Thus, diffusion anisotropy was lost in the paraventricular lesions. These findings are similar to those observed in the white matter lesions of cerebro-vascular origin. As a result, the pathology of the paraventricular lesions in ALS was confirmed to be different from that of the internal capsular lesions.

Diffusion MRI studies were performed on 14 patients with diffuse high-signal lesions of the cerebral white matter on T2-weighted MR images. There were two patients with adrenoleukodystrophy, four with dentato-rubro-pallido-luysian atrophy, three with familial spastic paraplegia, two with myotonic dystrophy and three with dementia of unknown origin. In addition, a patient with of Sanfilippo disease and one of the three patients who exhibited dementia of unknown origin were also found to be low-signal in the cerebral cortex T2-weighted MR images. In every case except the last two, diffusion-weighted MR images yielded significantly higher apparent diffusion coefficients perpendicular to nerve fibers in the frontal white matter and the corpus callosum than in the normal controls. The lesions displayed increased diffusion anisotropy, calculated as the ratio of the diffusion coefficient perpendicular to the nerve fibers to the diffusion coefficient parallel to the nerve fibers, i.e., diffusion anisotropy was lost in the white matter, suggesting a demyelinating process in the lesion. In the last two cases, on the other hand, the diffusion coefficient and diffusion anisotropy were within normal range. The white matter lesions in these cases were thought to represent degeneration secondary to the cortical involvement. Thus, it was confirmed that diffusion-weighted MR images, unlike T2-weighted MR images, were confirmed to allow differentiation between at least two types of white matter lesions.

FLAIR (fluid-attenuated inversion recovery) images are MR images obtained with an inversion recovery sequence having a long inversion time (TI) and a long echo time (TE). Twenty healthy adults and twenty patients with multiple cerebral infarction, multiple sclerosis, temporal epilepsy, or brain trauma were examined with FLAIR sequences of several types having repetitive times (TR) of 4000-8000 msec, inversion times of 1200-2400 msec and echo times (TE) of 140-200 msec, and the results were compared with spin-echo sequences (TE = 20 msec and TE = 90 msec). With a long repetitive time of 6000 msec and a long inversion time of 1400-1600 msec, the cerebrospinal fluid signals in the lateral ventricles and the cerebral sulci were low-intensity with brain tissue appearing as high signal intensity areas with good T2 contrast. The FLAIR image signal intensities correlated well with T2 relaxation times under 100 msec. Cystic lesions with long T2 relaxation times over 100 msec in multiple sclerosis and cerebral infarction appeared as low-signal areas, and the lesions surrounding the cystic lesions as high-signal areas. In patients with temporal lobe epilepsy, the hippocampus was visualized as a high-signal area. Hippocampal lesions were demonstrated better with FLAIR images than with conventional T2-weighted images or proton-density images. In a cerebral contusion patient, the FLAIR images revealed a lobulated structure with the residual cortex appearing as high-signal areas. The lesions surrounding the cystic change were imaged as high-signal areas. These structural changes were demonstrated better with FLAIR images than with conventional T2-weighted sequences.(ABSTRACT TRUNCATED AT 250 WORDS)

Clinical, radiological, and histological examinations were performed on eight patients with autosomal-dominant dentato-rubro-pallido-luysian atrophy (DRPLA) including an autopsy case, to investigate the abnormal findings of the cerebral white matter in DRPLA. Three of the eight patients were found to have diffuse low density on CT or diffuse high-signal areas on T2-MRI in the white matter of the brain. There were no correlations between abnormal findings in the white matter and the following factors; age of onset, duration of the disease, clinical manifestations, disease severity, Hachinski score, or EEG abnormality. Single-photon emission tomography failed to reveal any relative decrease in cerebral blood flow in the white matter, even in the three patients with abnormal findings in the white matter. MRI perfusion studies did not suggest any decrease in cerebral blood volume in any of the patients examined, but in the DRPLA patients the latency, i.e., the interval from the time of injection to the time of the minimum signal intensity, was significantly prolonged in comparison with the results in normal controls. On histopathological investigation, there was diffuse decreased staining in the centrum semiovale and deep white matter of the temporal lobes bilaterally, but no gliosis, or arteriolar thickening or hyalinization were detected. These findings confirmed that the lesions in the white matter in DRPLA are not attributable to cerebral ischemia. The abnormal findings are presumably the result of the degeneration which occurs in DRPLA itself.(ABSTRACT TRUNCATED AT 250 WORDS)

We analyzed diffusion weighted magnetic resonance images (diffusion MRI) of the basal ganglia, which were obtained from four patients with Wilson's disease, and compared them with the images from ten age-matched normal individuals. In all patients, T2-MRI of the basal ganglia disclosed low or iso-signals, but diffusion MRI revealed abnormal high signals in some areas of the basal ganglia in each case. Pathological changes except for copper and/or iron deposits are difficult to estimate by T2-MRI because the low signal on T2-MRI emphatically reflects the deposits, while the abnormal high signal on diffusion MRI is thought to reflect parenchymal lesions such as cell loss, demyelination and/or increase of the extracellular fluid. From our results, we confirmed that diffusion MRI was very useful for estimating parenchymal lesions with metal deposits.

MR imaging using the conventional spin each technique along with diffusion weighted imaging and water-fat imaging was performed in 16 patients with amyotrophic lateral sclerosis (ALS), 20 normal subjects, and 113 controls with other neurological disorders. Diffusion weighted images in the patients with ALS and the controls disclosed a high signal band from the subcortical area to the medullary pyramids. The high signal band on the diffusion weighted images corresponded to the pyramidal tract in the anatomical atlas described by Talairach. The T1- and T2-relaxation times, proton density, diffusion coefficient and diffusion anisotropy were measured at the points where high signal bands appeared on the diffusion weighted images. The T2-weighted images revealed high signal areas on the posterior limbs of the internal capsules in all the patients with ALS, 60% of the normal subjects, and 73% of the disease controls. The T1-weighted images disclosed high signal areas on the posterior limbs in 62% of the patients with ALS, but not in any of the normal subjects and the disease controls. The proton weighted images disclosed high signal areas on the posterior limbs in all the patients with ALS and 5% of the disease controls, but not in any of the normal subjects. Analysis of diffusion weighted images revealed no significant difference between the patients with ALS and the normal subjects in diffusion coefficient and diffusion anisotropy on the posterior limbs. Measurement of MR parameters (T1- and T2-relaxation times and proton density) showed that the proton density at the posterior limbs increased in ALS. Water-fat images using the method of Dixon revealed abnormal signals in the water images. These signal abnormalities were more prominent in the internal capsule than in the medullary pyramids. Our findings confirm that there is an increase in water molecules that have normal diffusion coefficient and diffusion anisotropy values in patients with ALS.

Serial examination of magnetic resonance images (MRI) for two months were carried out on two cases of multiple cerebral infarction during the acute stage. The T2-weighted MR images at the onset of the infarction showed both acute (new) and chronic (old) lesions appearing as high signal area. While on the diffusion weighted images only an acute lesion was detected as a high signal area with good contrast. The diffusion coefficient of the acute lesion was lower than that of normal white matter. Diffusion coefficient of the chronic lesions were higher than that of normal white matter. Therefore, on the apparent diffusion coefficient mapping images (ADC images) only an acute lesion appeared as a low signal area. The examination of diffusion images was very useful for distinguishing an acute lesion from a chronic lesion during the acute stage of multiple infarction. The diffusion weighted images after 4 weeks from the onset showed the diffusion coefficient of the "acute" lesion to be the same level of normal white matter. And after 8 weeks from the onset, increased to a level higher than that of normal white matter to the same level of the "chronic" lesion.

We reported a 55-year-old man, whose T2-weighted MR images disclosed abnormal high signal band along the left pyramidal tract 6 months after cerebral infarction of the left centrum semiovale. Brain CT revealed low intensity areas in the centrum semiovale, the posterior limb of the internal capsule on left side. On T2-weighted MR images, there were an irregular high signal area on the left centrum semiovale, a high signal band from the left centrum semiovale to the medullary pyramid, and a high signal band from the left centrum semiovale to the cerebral cortex. These lesions were observed as high signal areas on proton weighted images and low signal areas on T1-weighted MR images. Diffusion coefficient perpendicular to the pyramidal tract in the patient, which was calculated from diffusion weighted images at the posterior limb of the internal capsule, was higher than that in normal individuals. Diffusion anisotropy at the lesion, which is the rate between the diffusion coefficient parallel and perpendicular to nerve fiber, was higher than that of normal individuals. These data suggested that the lesion had demyelinating process, which was consistent with the pathology at stage 2 of the Wallerian degeneration.

We reported a 29-year-old woman with autoimmune polyglandular deficiency (APGD) type 1 accompanied by progressive myopathy. She had chronic mucocutaneous candidiasis at the age of 3, primary hypothyroidism at 12, insulin dependent diabetes mellitus at 27, and adrenal insufficiency at 29 years. Laboratory findings indicated an underlying defect in cell mediated immunity. Meanwhile, she had progressive muscular weakness and wasting at the age of 22 years which brought her to our hospital at 29 years. On admission, she could not walk without support and raise her arms up to the level of shoulders. Moderate to severe muscle wasting as well as weakness was observed in the limb girdle muscles. Serum CK levels were mildly elevated. A needle EMG examination disclosed short-duration and low-amplitude polyphasic motor units at voluntary contraction with few fibrillations and positive sharp waves at rest. On muscle CT examination, decreased density was detected in the neck extensor, paravertebral, rectus femoris, vastus intermedius, biceps femoris and soleus muscles. Muscle biopsy was performed on the biceps brachii and rectus femoris muscles. The former showed chronic dystrophic changes including marked variation in fiber size with necrotic and degenerating process, interstitial fibrosis, and lobulated and right fibers. In the latter, in addition to variation in fiber size with some necrotic fibers and occasional multi-core structures, nemaline bodies were seen in approximately 30% of muscle fibers. The progressive muscle involvement in our patient might be induced from 1) endocrine abnormality, 2) autoimmune disorder, and/or 3) coincidental complication of nemaline myopathy or limb girdle muscular dystrophy. The clinical and laboratory examinations, however, failed to support any of them.(ABSTRACT TRUNCATED AT 250 WORDS)