A patient with splenic marginal zone lymphoma presenting with spastic paraplegia as the initial symptom.

In this report, we describe the case of a patient with splenic marginal zone lymphoma (SMZL) who presented with spastic paraplegia as the initial symptom. A 42-year-old male developed progressive spastic paraplegia over 4 months. His neurologic examination revealed paraplegia with pyramidal syndrome, hypoesthesia below the T1 level, and anal hypotonia. Magnetic resonance imaging (MRI) of the spinal cord revealed an extensive high-intensity signal in T2-weighted sequences and swelling involving the thoracic region and conus medullaris. A laboratory test revealed presence of the serum M component. Abdominal computed tomography images showed moderate splenomegaly. Abnormal lymphocytes of B-cell lineage markers (CD19+, CD20+, and CD25+; surface immunoglobulin κ expression; IgD+ and IgM+) were found in the peripheral blood, cerebrospinal fluid, bone marrow and spleen. Splenectomy confirmed the SMZL diagnosis. After the completion of chemotherapy, the patient was in complete remission, and spinal MRI findings were normal. Intramedullary spinal cord involvement in SMZL is extremely rare, and, to the best of our knowledge, this is the first case of SMZL with intramedullary spinal cord involvement associated with clinical and radiologic signs without the involvement of cerebral structures. Spastic paraplegia can be the initial presentation of SMZL.

Paradoxical cerebral embolism as the initial symptom in a patient with ovarian cancer.

This report concerns a 37-year-old patient with ovarian cancer and a paradoxical cerebral embolism as the initial symptom. She developed acute onset of left quadrantic hemianopia during coughing. Brain magnetic resonance imaging showed an acute multiple infarction, and a simultaneous acute pulmonary embolism was observed. Transesophageal echocardiography showed a patent foramen ovale, multidetector row computed tomography an ovarian tumor and infarction of the spleen, whereas multidetector row computed tomography venography showed right iliac vein compression by the ovarian tumor. The diagnosis was stage Ic ovarian cancer. Because blood stasis of the pelvic vein is a major risk factor for venous thrombosis, the presence of a patent foramen ovale should alert physicians to examine not only veins in the lower extremities but also the pelvic and intra-abdominal veins as a source paradoxical embolism.

[Successful use of methylprednisolone therapy in a case of non-herpetic acute encephalitis with antibodies against ionotropic glutamate receptor epsilon2 and delta2].

This report concerns a 32-year old man with non-herpetic acute limbic encephalitis. He was admitted to our hospital because of high fever and consciousness disturbance. Cranial MRI revealed abnormal signal intensities in the bilateral mesial temporal lobes. On the second hospitalization day, he developed status epilepticus, which necessitated general anesthesia. Following administration of intravenous (IV) methylprednisolone pulse therapy (1 g/day, 3 days), his consciousness disturbance began to improve. Treatment with high-dose IV methylprednisolone was continued for about 2 weeks, but on the 7th day, he showed severe anterograde amnesia and retrograde amnesia regarding the preceding 5 or 6 years. Subsequently, however, his amnesic disorders improved markedly, and on the 46th day, memory dysfunction had disappeared. Autoantibodies against the glutamate receptor subunits epsilon2 and delta2 were detected in both the CSF and serum, but these antibodies in the CSF became normal during the clinical course. The voltage-gated potassium channel antibody was negative. This case report indicates that high-dose IV methylprednisolone therapy may be an affective treatment for non-herpetic acute limbic encephalitis, possibly associated with autoimmune mechanisms.

[A patient with primary Sjögren's syndrome featuring polyneuropathy and oculomotor paralysis and associated with asymptomatic left middle cerebral artery stenosis].

We report a case of primary Sjögren's syndrome (primary SjS) with polyneuropathy and right oculomotor paralysis associated with middle cerebral artery stenosis. A 39-year-old woman developed progressive numbness and clumsiness of the limbs. Two months later, right third cranial palsy manifested itself and she was admitted to our hospital. A cranial MRA showed left middle cerebral artery stenosis confirmed by transcranial color doppler sonography. A nerve conduction study showed a decrease in the NCV and reduced CMAP, while sural nerve biopsy showed axonal degeneration and infiltration of inflammatory cells around the small blood vessel walls. The patient complained of dry mouth and a salivary gland biopsy revealed inflammatory changes, while salivary gland scintigraphy showed diminished secretion. These findings led to the diagnosis of Sjögren's syndrome. Reports of primary SjS with involvement of large cerebral arteries are rare. In our case, polyneuropathy and oculomotor paralysis were the manifest symptoms, but middle cerebral artery stenosis was also observed. This indicates that, even in the absence of CNS symptoms, cerebral artery involvement may be present in primary SjS.