[A case of adult onset subacute sclerosing panencephalitis; sequential clinico-radiological findings over two years].

We present a very rare case of adult onset subacute sclerosing panencephalitis (SSPE), and explain the characteristic sequential clinico-radiological findings. The patient, a twenty three-year-old man, had noticed unsteadiness in walking about two months previously. Although inosine pranobex and intrathecal interferon were administered, symptoms worsened insidiously and he became bedridden after four months. The levels of serum and CSF anti-measles IgG antibodies have not changed. Initially, supratentorial cortical atrophy was observed, especially of the left temporal lobe, but there were no other MRI signal alterations at the time. After three months, the supratentorial cortices produced low-signal intensities in T1-weighted images and in T2-weighted MRI, the cortical margin was very unclear and white matter signal intensities had become higher. Furthermore, cortices became thinner and ventricular size increased, especially for the lateral and third ventricles. SPECT examinations showed a marked reduction in cerebral blood flow and the perfusion deficits observed seemed to be closely correlated with the abnormal MRI signal patterns. Pathological examinations of biopsy samples revealed infiltration of inflammatory cells around the small vessels. As for immunohistochemical findings, CD68 positive cells were frequently observed, and this result implied the activation of microglia. Further studies are necessary to elucidate the pathogenic mechanisms of SSPE.

Primary CD30/Ki-1 positive anaplastic large cell lymphoma of the central nervous system occurring in a patient with a seventeen-year history of essential thrombocythemia.

We present a rare case of central nervous system lymphoma that occurred in a patient who had essential thrombocythemia for 17 years. MRI examinations disclosed multiple ring-enhanced lesions that had shown bilateral spreading in the different period. Pathological examinations confirmed CD30/Ki-1-positive ALK negative anaplastic large cell lymphoma. The possible pathogenic mechanisms of this disease are discussed.

[Two cases of hypertrophic cranial pachymeningitis associated with infection in the external auditory canal and paranasal sinus].

We here present two cases of hypertrophic cranial pachymeningitis exhibiting unique multiple cranial neuropathies, both of which were associated with otic and paranasal infections. Case 1: A 76-year-old woman developed headache after undergoing surgical dilatation of the external auditory canal, with subsequent development of a bacterial infection. Neurological examination reveled only bilateral hearing disturbance. MRI and CT scans demonstrated thickening of the dura mater and inflammatory granulation around the left cerebellar tentorium. Based on a diagnosis of hypertrophic pachymeningitis associated with previous infection, antibiotics were administered, followed by oral prednisolone therapy. This treatment relieved the headache and improved the MRI findings. However, 2 months later, the headache became worse and impaired movement of the soft palate, atrophy of the left side of the tongue, and atrophy of the sternocleidomastoideus muscle were noted. MRI revealed aggravated inflammatory changes around the left cerebellar tentorium and their expansion into the jugular foramen. Occlusive changes in the transverse and sigmoid sinuses were also seen. Case 2: A 78-year-old man developed bilateral visual loss, right frontal headache, and bilateral restriction of eye movement. He had been treated for phemphigus with prednisolone and azathioprine. MRI showed hypertrophic dura mater spreading continuously from the frontal base and ethmoid and frontal sinuses to the falx and right frontal lobe. Since Pseudomonas aeruginosa was cultivated in biopsy specimens from the dura mater, antibiotic agents were administered. The clinical symptoms resolved and MRI findings gradually improved.

[Intracranial hypertrophic pachymeningitis with high perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA) occurred in a patient on hemodialysis].

We present a 66-year-old woman undergoing hemodialysis who developed intracranial hypertrophic pachymeningitis. Neurological examinations revealed a loss of bilateral visual acuity with optic atrophy, headache, and markedly restricted bilateral extraocular movement. MRI examinations demonstrated homogenous hypertrophic dural enhancement compatible with hypertrophic cranial pachymeningitis, and biopsied dural specimen revealed chronic inflammatory changes with proliferation of dense collagen fibers. There was no direct evidence of vasculitis and specific infections including tuberculosis and troponema pallidum. Most of the inflammatory infiltrates were demonstrated to be T lymphocytes. Intriguingly, p-ANCA was found to be highly elevated at x 220 and decreased to x 110 after steroid treatment. Neurological manifestations and radiological findings also improved in accordance with the lowering of p-ANCA. Although a few reports have described similar conditions such as chronic renal failure accompanying hemodialysis and pachymeningitis, and though vasculitis was not depicted histologically in this patient, we considered that immunological mechanisms probably provoked the patient's glomerulonephritis and pachymeningitis. Additionally, positive reaction against hepatitis c virus might have influenced the immunological system leading to the occurrence of the pachymeningitis.