Yasutaka Tajima

Detection of a novel HLA-DQ specificity: serological and immunochemical analyses by a monoclonal antibody

A monoclonal antibody (mAb) with a novel human B-cell allospecificity was produced by immunizing a C3H/He mouse with the human B lymphoblastoid cell line EBV-Wa (HLA-DR4/Dw15/DQblank homozygous). The mAb, termed HU-46, reacted with B cells from not only DR4/Dw15-positive individuals but also certain DRw8/Dw8-positive ones whose DQ phenotypes had not yet been defined. Two-dimensional gel analyses indicated that the mAb recognized class II antigens which were encoded by the HLA-DQ locus. Furthermore, in genetic analysis, the gene encoding the class II antigen detected by HU-46 met the Hardy-Weinberg condition as a fourth allele of the DQ locus. We provisionally labeled this novel DQ specificity DQWa.

Clinical features of spinal cord sarcoidosis: analysis of 17 neurosarcoidosis patients

The diagnosis of neurosarcoidosis is often difficult; the imaging signs of spinal cord sarcoidosis sometimes mimic those of cervical spondylotic myelopathy, which is common in elderly persons. We examined the characteristics of spinal cord sarcoidosis in Japanese patients with neurosarcoidosis. This case series identified patients with neurosarcoidosis at four general hospitals and one university hospital from April 1998 to September 2010. All diagnoses were based on the diagnostic criteria proposed by Zajicek et al. Seventeen patients (nine men and eight women) were involved: six patients with spinal cord lesions accompanied by cervical spondylosis, five with cerebral lesions, three with cranial nerve lesions, two with meningitis, and one with nerve root lesions. Patients with spinal cord sarcoidosis had a higher onset age, longer duration from onset to diagnosis, reduced leukocytosis in the cerebrospinal fluid (CSF), and lower angiotensin-converting enzyme (ACE) levels in the CSF. The results of this study indicate that diagnosis of spinal cord sarcoidosis requires careful evaluation.

UNUSUAL RENAL PELVIC TUMOR CONTAINING TRANSITIONAL CELL CARCINOMA, ADENOCARCINOMA AND SARCOMATOID ELEMENTS (SO-CALLED SARCOMATOID CARCINOMA OF THE RENAL PELVIS)

A case of sarcomatoid carcinoma of the renal pelvis in a 66‐year‐old male is reported. The patient underwent left nephroureterectomy because of renal pelvic tumor and hydronephrosis. Left renal calcification and atrophy had been diagnosed in the patient about thirty years previously. The tumor showed a polypoid configuration and occupied the renal calyces. Histologically, not only solid nests of transitional cell carcinoma (TCC) and adenocar‐clnomatous glands but also large spindle‐shaped cells with bizarre nuclei simulating sarcoma were identified. Immunoreactlve keratin and epithelial‐membranous antigen (EMA) were demonstrated in the sarcomatoid cells, indicating that they were of epithelial origin. So far, only 10 cases of so‐called sarcomatoid carcinoma or carcinosarcoma of the renal pelvis have been reported in the world. In this report, we summarize the pathological findings of previously reported cases and discuss the histogenesis of this rare tumor.

MR Appearance of Subacute Combined Degeneration of the Spinal Cord

Abstract: Subacute combined degeneration (SCD) of the spinal cord is well known to produce degenerative lesions in the spinal cord histopathologically, but a few reports on the neuroradiological findings have so far been reported. We presented the MRI findings of the spinal cord in a case of SCD. The localization of the radiologically proven lesions was similar to that of the previous pathological reports.

Spinal Magnetic Resonance Image alterations in human T-lymphotropic virus type I-associated myelopathy patients before and after immunomodulating treatments.

Sirs: Previous reports have been that most of the spinal cord lesions of Human T-lymphotropic virus type I associated myelopathy (HAM) were atrophy induced by the chronic inflammatory processes, and that these changes seldom showed alterations in MRI signal intensity [1–3]. In contrast, we present here two cases of HAM patients in whom the spinal cord showed moderate swelling both with gadolinium-DTPA enhancement and faint high T2 signal intensities, and in whom these alterations were partially resolved after interferon-alpha and steroid treatments. Case 1: A 66-year-old woman noticed paresthesia in her left arm and walking difficulty about eight months prior to admission. Suspected of having cervical and thoracic myelopathy, she underwent decompression of the cervical and thoracic spine at another hospital. Her neurological symptoms did not significantly improve, and in fact, there was gradual worsening. Neurological examination revealed marked spasticity of the lower extremities, and slight spasticity of the upper limbs. Deep tendon reflexes were hyperactive, and pathological reflexes were easily elicited. Serum antibody for HTLV-I was elevated to x8132, as was that of CSF x32. Additionally, Western blot analysis demonstrated P19 and p24 in the CSF. Protein in the CSF was elevated to 55 mg/dl, and IgG was 7.7 mg/dl (normal; 4 mg/dl >). No pleocytosis was seen (3/mm3). Both oligoclonal bands and MBP (myelin basic protein) were negative. On the basis of these findings, the patient was diagnosed as having HAM. Previous T2-weighted MRI performed prior to operation demonstrated high signal intensity in the patient’s cervical and thoracic cord (Fig. 1A,B). Moreover, the spinal cord showed faint Gdenhancement. Cranial MRI did not show white matter lesions, which are well known to be associated with HAM. As the initial treatment, we administered three million units of interferon-alpha intramuscularly daily for twenty-eight days. Following the interferon treatment, pulsed steroid therapy was employed, and oral steroid dose was gradually tapered. Then, the frequency of the tonic spasms in the lower extremities decreased. Intriguingly, MRI showed that T2 high signal intensity and gadolinium-DTPA enhancement of the spinal cord had almost disappeared (Fig. 1C,D). Case 2: A 51-year-old woman was transferred to our department because she had had walking difficulty and dull pain over both her lower extremities for about four months prior to admission. Neurologically, the spasticity of her extremities was prominent, and deep tendon reflexes were markedly hyperactive. Pathological reflexes were easily elicited. She also showed a markedly unsteady gait. Her serum anti-HTLV-I antibodies were highly elevated (x16384). Since her CSF examination had also revealed a high titer of HTLV-I antibody (x1024), and Western blotting had demonstrated p19 and p24 proteins, we concluded that she was suffering from HAM. MRI demonstrated cervical and thoracic cord swelling with faint T2 high signal intensities (Fig. 2A,B,C). Additionally, gadolinium-DTPA enhancement in the cervical cord revealed high signal areas in the lateral columns, which resembled “owl eyes” (Fig. 2D). Cranial MRI was regarded as normal. The spinal fluid showed slightly elevated protein 46 mg/dl and mild pleocytosis (33/mm3). IgG in the CSF was 6.7 mg/dl (normal; 4.0 mg/dl >). Neither oligoclonal bands nor MBP were found. The patient was initially administered steroid (1 mg/kg), and the dose was gradually tapered. In addition to the steroid treatment, interferon-alpha was administered every day for twenty-eight days. Her spasticity appeared to decrease, DTR became slightly hyperactive, and bilateral pathological reflexes showed weakly positive. Her walking speed increased, and a steadier gait was seen. MRI revealed a less swollen spinal cord, and the previously observed T2 high signal in the cervical cord had disappeared (Fig. 2E). Despite the improvement in cord swelling, T2 high signal intensities within the thoracic cord remained (Fig. 2F). In spite of intensive investigation including auto-antibody analysis, systemic CT and scintigrams, no other systemic disorders which could generate myelopathies were found in either case. However, we cannot completely exclude the possibility that protruding cervical disks at C5/6 and C6/7 in case1 and LETTER TO THE EDITORS

Hot water epilepsy with pineal cyst and cavum septi pellucidi.

Abstract: A case of reflex epilepsy accompanied by pineal cyst and cavum septi pellucidi induced by hot water bathing is presented. The patient is a 25‐year‐old male who has had six episodes of convulsions during the last three years. The seizures were diagnosed as a complex partial epilepsy followed by generalized tonic seizures. We have succeeded in recording his electroencephalogram (EEG) during convulsions. Moreover, a magnetic resonance imaging (MRI) study revealed a pineal cyst and cavum septi pellucidi. This is quite a rare form of adult hot water epilepsy accompanied by intracranial malformations.

Cranial neuropathy because of IgG4-related pachymeningitis; intracranial and spinal mass lesions.

A 64-year-old man suffering from chronic heart failure developed gradually worsening dysphagia 1 month before. Physical examinations revealed no abnormalities, such as proptosis and parotid swelling. Neurological examinations revealed hoarseness, poor soft palate elevation and right-sided atrophy of the tongue and sternocleidomastoid muscle. The patients DTRs were slightly hypoactive, but no pathological reflexes were elicited. Cerebellar systems were intact, …

Assignment of the possible HTLV receptor gene to chromosome 17q21-q23

We have determined the HTLV (Human T-cell leukemia virus) receptor localization more precisely on the human chromosome 17. Based on the fact that HTLV infection induces syncytium formation of infected cells as a result of interaction between the viral envelope and viral receptor, we performed the sensitive biological assay using recombinant vaccinia expression system. Our results from the induced syncytium pattern of the somatic hybrid cell lines with different deletions indicated that the HTLV receptor gene may reside from q21 to q23 on the long arm of the human chromosome 17.

Unusual basal ganglia lesions in a diabetic uraemic patient proven to be demyelination: first pathological observation

A 64-year-old man suffering from diabetes mellitus and chronic renal failure was admitted to our hospital because of consciousness disturbance and parkinsonism. Cranial MRI showed very characteristic features involving the bilateral basal ganglia. Subsequent postmortem examinations demonstrated demyelination in the affected areas. These myelin destruction patterns were quite similar to those of central pontine myelinolysis. However, rapid correction of hyponatraemia was ruled out in this patient. Therefore, a new demyelinating brain disease associated with diabetes mellitus and chronic renal failure was suggested.

Detection of a novel HLA-DQ specificity

At the Ninth International Histocompatibility Workshop (Munich, 1984), DP, DQ, and DR antigens were officially recognized as the class II gene products of the H L A complex (Bodmer et al. 1984). The DP, D Q , and D R loci were considered to have at least 6, 3, and 14 (2 more in a proximal locus) alleles, respectively. DQ molecules have been revealed to be the human homolog of mouse A molecules (Bono and Strominger 1982, Giles et al. 1983), although only three alleles have been identified despite the fact that the mouse A system is highly polymorphic (Klein et al. 1983). Recent findings of twodimensional gel analyses, however, have demonstrated that the D Q system is as polymorphic as the mouse A system (de Kretser et al. 1982, Shackelford et al. 1983, Maeda et al. 1984, Ishikawa et al. 1985). Nevertheless, no serological reagent was available to detect the fine specificity corresponding to these observations, except for a few mouse monoclonal antibodies (mAbs) such as TA10 and HU-23, both of which dissected DQw3 antigens associated with Dw4 and Dw5 from those associated with other D specificities (Kasahara et al. 1983, Maeda 1984). Thus, only three kinds of alloantisera detecting the supertypic determinants on DQ molecules were utilized to define the phenotypes of the DQ antigens as DQwl, w2, and w3. Moreover, the limitation of antisera made it difficult to define the DQ antigens associated with the Dwl5-DR4 and certain Dw8-DRw8 specificities, which do not belong to any of the DQw 1, w2, and w3 families (Schreuder and Degos 1984). To investigate the D Q system, we produced mouse mAb HU-46, which defines a novel DQ specificity,