One explanation for cerebral infarctions that occur as a complication of pneumococcal meningitis is blood coagulation abnormalities. We investigated the clinical features, laboratory test results, magnetic resonance imaging (MRI) findings, and pathological features of 10 patients with pneumococcal meningitis between 2006 and 2013 to examine the abnormal findings that may be associated with prognosis. Five patients (50%) that had Glasgow Outcome Scale scores between 1 and 4 were classified as the poor outcome group. In this group, the MRI revealed a high signal intensity on the diffusion-weighted image (DWI), and there was an abnormal signal along the cerebral cortex and Virchow-Robin spaces, which were characterized pathologically by ischemic changes. The plasma thrombin-antithrombin complex (TAT) levels showed greater differences between the poor and good prognosis groups than platlet and D-dimer levels; this suggested that high plasma TAT levels indicate a poor prognosis.

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The patient was a 78-year-old man. Three years before admission, he developed transient peripheral neuropathy and purpura, and at admission, he presented with livedo reticularis of both his lower extremities and with mononeuritis multiplex. Vasculitis was not observed, and antiphospholipid antibodies were detected. The nerve and skin biopsies revealed no inflammation; axonal degeneration accompanied by thrombi was found in his arterioles and venules. Based on these findings, he was diagnosed with ischemic peripheral neuropathy due to primary antiphospholipid syndrome. Administration of anticoagulant therapy resulted in an improvement in symptoms; however, two months later, a relapse occurred, and the patient contracted an infection while undergoing immunosuppressive therapy. The infection became fulminant, and the patient succumbed to multiple organ failure. The autopsy revealed a systemic arterial and venous embolism; however, no vasculitis was observed. Antiphospholipid syndrome, which is responsive to antithrombotic treatment, should be considered as a differential diagnosis of mononeuritis multiplex.

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A 69-year-old woman presented with non-fluent aphasia, ideomotor apraxia, right hemiparesis and convulsion. Her medical history was unremarkable, and she had not suffered from arthritis. DWI and FLAIR image of brain MRI showed hyperintensities in the subarachnoid space along the left frontal and both parietal lobes, and these lesions were associated with gadolinium enhancement. The levels of serum anti-cyclic citrullinated peptide antibody, anti-agalactosyl IgG antibody and matrix metalloproteinase-3 were elevated. The results of blood cultures were negative. Cerebrospinal fluid (CSF) analysis revealed monocytic pleocytosis and negative findings for infection or malignancy. The level of anti-agalactosyl IgG antibody in CSF was elevated. The antibody index (AI) of anti-agalactosyl IgG antibody (the ratio between the CSF/serum quotient for IgG antibodies, and the CSF/serum quotient for total IgG; normal value of AI < 1.3) showed considerably high value of 8.4, indicating the intrathecal-specific antibody synthesis. As a result, the pathogenesis of her disease was consistent with rheumatoid meningitis despite lack of arthritis. After intravenous administration of methylprednisolone, her symptoms, the level of anti-agalactosyl IgG antibody in CSF, and the MRI findings were ameliorated. Anti-agalactosyl IgG antibody in the CSF was a helpful biomarker in diagnosis and assessment of the severity of rheumatoid meningitis.

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A 23-year-old man presented tonic-clonic seizure a week after an episode of antecedent infection. Although several anticonvulsants were used, convulsive attacks were not resolved and intravenous anesthetics were used to stop status epileptics. After combination of immunotherapies (high-dose intravenous methylprednisolone, immune absorbance and intravenous immunoglobulin (IVIg) therapies), frequency of convulsive attacks decreased, however, disturbance of consciousness was not recovered. All anti-neuronal antibodies tested were negative. Indirect immunofluorescence using the serum and rat brain section revealed positive signals in cytoplasm and nucleus in hippocampal neurons, strongly suggesting that this case has an autoimmune pathogenesis. The clinical features and course of this patient are well consistent with those in new-onset refractory status epilepticus (NORSE). The result of immunohistochemical analysis supports the hypothesis that NORSE has an autoimmune pathomechanism.

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In this communication, we report a patient with familial amyotrophic lateral sclerosis (ALS) associated with a familial dyslipidemia. Genetic analysis revealed a novel heterozygous valosin-containing protein (VCP) mutation (c.466G>T (p.G156C)). The other gene analysis also disclosed a known homozygous LCAT mutation (c.101C>T (p.P10L)). VCP gene mutation shown should be responsible for familial ALS because of following reasons. The patient’s father also was also affected by ALS. The VCP gene mutation (p.G156C) in the patient was located in the vicinity of a site frequently associated with pathogenic VCP variants. The same amino acid transformation as that of this patient has been reported to be involved in the pathogenesis of inclusion body myopathy with Paget’s disease of the bone and frontotemporal dementia. This is the first case report of rare association of ALS with VCP mutation and dyslipidemia with LCAT mutation.

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A 69-year-old man experienced general malaise, weight loss, amnesia, gait disturbance, and restlessness a month prior to admission. Brain MRI showed high intensity areas in the bilateral medial temporal lobes and insular cortices on FLAIR images, and therefore, he was diagnosed with limbic encephalitis. After admission, quadriplegia and respiratory failure progressed rapidly, and he needed ventilatory management. A nerve conduction study revealed low compound muscle action potential amplitude with loss of sensory nerve action potential, which indicated axonal sensorimotor neuropathy. We administered intravenous immunoglobulin and methylprednisolone pulse therapy, but he did not recover. Although no tumor was found on CT, his serum was positive for anti-Hu antibody; therefore, we diagnosed him with paraneoplastic neurological syndrome. An FDG-PET study showed accumulation at lesions on two hilar lymph nodes. Small cell lung carcinoma was detected by endobronchial ultrasound-guided transbronchial needle aspiration. Although paraneoplastic acute sensorimotor neuropathy with respiratory failure resembling Guillain-Barré syndrome is rare, identification of antibodies and servey of tumors aids accurate diagnosis.

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We report a 65-years-old woman with rheumatoid meningitis presented with a generalized seizure. She has a 18-year history of rheumatoid arthritis, which has been successfully treated. She developed a generalized seizure. She was diagnosed as having subarachnoid hemorrhage, because the brain magnetic resonance imaging (MRI) showed increased fluid attenuated inversion recovery (FLAIR) signals in her left frontoparietal subarachnoid space. After one month of clinical stabilization, she developed numbness and weakness in her right lower extremity that spread to her right upper extremity and face. Brain MRI showed progression of subarachnoid lesion on FLAIR image and leptomeningeal enhancement on gadolinium-enhanced T₁ weighted image. She was diagnosed as having rheumatoid meningitis, and methylprednisolone pulse therapy was started. Then, her symptoms and MRI findings were rapidly improved. Though rheumatoid meningitis is rare and presents a difficulty in the diagnosis, MRI features may support the diagnosis.

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A 62-year-old woman developed meningitis as well as acute paralysis of glossopharyngeal, vagus, and accessory nerves on the right side and also had dysfunction of the left hypoglossal nerve. Although there was no evidence of a typical cutaneous or mucosal herpetic lesion, PCR detection of varicella zoster virus (VZV)-DNA in cerebrospinal fluid confirmed the clinical diagnosis of polyneuritis cranialis due to VZV infection and zoster sine herpete. After starting intravenous acyclovir and methylprednisolone, her hypoglossal nerve palsy disappeared within a day and all other symptoms and signs dramatically improved. A rapid improvement observed in our patient suggests that the right cranial polyneuropathy could be caused by inflammation associated with epineurial edema (where the ninth, tenth, and eleventh cranial nerves pass through the right jugular foramen), whereas the exact mechanism of the twelfth cranial nerve involvement on the contralateral side is unknown. Our clinical findings indicate that acute lower cranial polyneuropathy in patients with zoster sine herpete should be treated immediately with combined administration of acyclovir and an anti-inflammatory corticosteroid.

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This report describes a 15-year-old woman presenting posterior reversible encephalopathy syndrome (PRES) due to azathioprine. She was under treatment for ulcerative colitis. She was prescribed azathioprine seven days before admission. Four days after, she complained of headache. Then, she disturbed consciousness and showed generalized convulsive seizure on the day of admission. Magnetic resonance imaging (MRI) revealed vasogenic edema in both hemispheres. She was discontinued azathioprine and treated by anticonvulsant. Her symptoms were recovered and the MRI findings were disappeared. We diagnosed as PRES due to azathioprine because of clinical course and MRI findings. Only one case of PRES due to azathioprine is reported previously. Our case is the first report that showed the changes and improvement of MRI findings along the clinical course.

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A 71-year-old woman was referred to our department for evaluation of a right temporal headache. She had been diagnosed with Takayasu arteritis in her twenties but did not receive steroid therapy. A brain MRI scan detected thickened dura mater with abnormal enhancement on the right cerebral hemisphere. She was diagnosed with hypertrophic pachymeningitis, but she refused to be treated with steroids. Three months later, she noticed periorbital pain and blurred vision in her left eye, although the right temporal headache was reduced. A brain MRI scan detected thickened dura mater with abnormal enhancement on the left cerebral hemisphere adjacent to the left orbit and a swelled left superior rectus muscle with abnormal enhancement. However, the MRI results also showed that the thickening of the dura mater on the right cerebral hemisphere had improved. The new symptoms and the abnormalities revealed with imaging were resolved following steroid therapy. Takayasu arteritis mainly affects the large vessels, but can involve small and systemic vessels. This case presents a rare but possible link between hypertrophic pachymeningitis and Takayasu arteritis.

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[in Japanese]

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[in Japanese]

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