[A 34-year-old woman with cat scratch disease who developed encephalopathy].

We experienced a patient with cat-scratch disease (CSD) who developed encephalopathy. The subject was a 34-year-old female who had been aware of a low-grade fever and swollen inguinal lymph nodes for 1 month. The subject's consciousness became impaired after a headache developed, accompanied by fever, nausea, and vomiting, and she subsequently sought medical consultation. No major abnormalities were observed in the cerebrospinal fluid and cranial magnetic resonance imaging (MRI) was normal. However, necrotizing lymphadenitis was observed on contrast enhanced computed tomography (CT) of the pelvis and granulomatous changes were observed in a surgical lymph node biopsy. As the subject lived with a pet cat, PCR testing for Bartonella henselae (the CSD pathogen) was performed using a tissue biopsy. This was positive and the subject was diagnosed with CSD encephalopathy. There are very few domestic reports of CSD encephalopathy and care must be taken not to overlook this disease.

[Case of primary intraocular central nervous system lymphoma with high interleukin 10 level and positive cytology in cerebrospinal fluid].

A 73-year-old woman was admitted to the surgical department of our hospital for endoscopic resection of a colonic polyp. The day after endoscopic resection, she became drowsy and dysphasic. Two days later, left hemiparesis and gait difficulty developed. The next day, hemiparesis progressed bilaterally and dyspnea developed due to upper airway stenosis. The most prominent signs were those of bulbar palsy. Blood analysis revealed mild inflammatory responses and hyponatremia. T2-weighted magnetic resonance imaging showed high-intensity lesions in the swollen medulla and cervical spinal cord. Those areas and the meninges of the posterior fossa were enhanced by gadolinium. Steroid pulse therapy was administered, resulting in rapid recovery of bulbar and paretic symptoms with decreased enhanced area. At this point, concentration of cerebrospinal fluid interleukin (IL)-10 was markedly elevated at 146 pg/ml (normal,< 5 pg/ml), suggesting malignant lymphoma. Cytology of the cerebrospinal fluid was repeatedly examined, eventually revealing atypical lymphocytes with hyperlobulated nuclei and clear nucleoli. Lymphocytes stained with anti-CD20 antibody. These findings strongly suggested a diagnosis of primary intraocular and central nervous system lymphoma. In the present case, repeated cytology of cerebrospinal fluid was highly important for diagnosis in this case of high IL-10 level in cerebrospinal fluid.

[The painful multiple mononeuropathy of acute onset in the left arm which was diagnosed as leprous neuropathy].

A 31-year-old man from Myanmar with leprous neuropathy was reported. The progress of the disease was subacute but the painful symptom at the time of the onset was acute. Multiple mononeuropathy was diagnosed by the biopsy findings of the left superficial radial nerve. He was admitted to our hospital with the complaint of the weakness of his left hand and fingers which were very painful and got worse in several weeks. Motor palsy was observed in his left ulnar, median, and radial nerves, and there was the hypesthesia or anesthesia in his left hand, forearm and the medial side of his left upper arm. On nerve conduction studies, the amplitudes of CMAP and SNAP severely diminished or not detected. The pattern was compatible with multiple mononeuropathy. The biopsy of the left superficial radial nerve was performed. The pathological findings were the destruction of nerve fascicles, replacement of nerve fibers with inflammatory cells, and Mycobacterium leprae was found with the specific stain. These findings confirmed the diagnosis of the leprous neuropathy. Leprous neuropathy is one of the commonest causes of infectious neuropathy in the world, especially in Southeast Asia. These days many foreign workers from that area are staying in Japan, and the chances to see the disease are increasing. We have to recognize leprous neuropathy as a candidate for the multiple mononeuropathy of acute onset with painful dysesthesia similar to vascular neuropathy.

[Overlap syndrome of systemic lupus erythematosus and dermatomyositis presented a large demyelinating subcortical lesion mimicking brain tumor and high level of CSF antineuronal and serum anti-ribosomal P antibodies].

We reported a 50-year-old man with an overlap syndrome of dermatomyositis and SLE, whose magnetic resonance image of the brain showed a rapidly increasing large tumor-like focal lesion unequally enhanced by Gd-DTPA in the left frontal lobe. Its pathological finding by the brain biopsy was fibrinoid necrosis, inflammatory cell aggregation around blood vessels and many myelin-laden macrophages with central necrosis. Although many cases of blood vessel injury are reported in CNS lupus, in this case the brain lesion partly took reversible course and neural symptoms such as paresis were slight and the lesion well responded to steroid. Moreover we considered that the measurement of serum anti-ribosomal P and CSF antineuronal antibodies are useful to diagnose cases as CNS lupus.