[Role of the liaison officer in disaster countermeasures implemented by the Japanese Society of Neurology: Hope for the best and prepare for the worst].

Disaster countermeasures have been implemented by the Japanese Society of Neurology based on the experience of support to the areas affected by the Great East Japan Earthquake on March 11, 2011. The countermeasures activity began at the end of 2011. We, the Committee for Measures Against Disaster, officially started work in 2014. We developed a support network to urgently deal with patients with intractable neurological disease at the time of disaster and strengthen disaster measures, including effective disaster countermeasure training. During the 2016 Kumamoto earthquake, we realized the need to prepare for natural disasters, leading to a state of emergency, at normal times. A list of vulnerable people should be prepared and the individual support plan for disaster should be confirmed during normal times. Furthermore, during disaster, livelihood support is required for patients with intractable neurological disease living in evacuation centers in affected areas. Therefore, we compiled and published the book, titled "The manual of disaster countermeasures," in 2017. The Committee for Measures Against Disaster in the Japanese Society of Neurology has appointed a liaison officer for patients with intractable neurological disease in each prefecture. The liaison's role of is gathering and disseminating information on the disaster-hit areas, arranging medical support, and coordinating health activities, when natural disasters occur. It is hoped that the liaison officer will play an active role both at normal times and during disaster, even unforeseen ones. Although we hope for the best, we aim to be prepared for the worst.

Prognostic value of cerebrospinal fluid cytokine changes in herpes simplex virus encephalitis.

A recent trial suggested that corticosteroid was beneficial in herpes simplex virus encephalitis (HSVE), but that precise role remains unclear. We assessed the differences of cerebrospinal fluid (CSF) cytokine changes between different outcomes and between patients with and without corticosteroid administration at the acute stage of HSVE. Interleukin (IL)-1beta, IL-2, IL-6, IL-10, interferon (IFN)-gamma, and tumor necrosis factor-alpha were measured in 56 serial CSFs taken from 20 adult HSVE patients. Their outcomes were poor in 7 and good in 13 patients, and corticosteroid was administered in 10. The differences in the initial and maximum cytokine values were assessed among the different outcomes. The decline rate of cytokine values between the initial and second CSF samples was also assessed between patients with and without corticosteroid. The initial IFN-gamma and maximum IL-6 with a poor outcome were higher than those with a good outcome (p=0.019 for IFN-gamma and p=0.013 for IL-6). The decline rate of IL-6 in patients with corticosteroid was higher than that without corticosteroid (p=0.034). The initial IFN-gamma and maximum IL-6 CSF values represented prognostic biomarkers in HSVE. One pharmacological mechanism related to corticosteroid in HSVE is apparently inhibition of pro-inflammatory cytokines such as IL-6.

[Acute sensory neuropathy associated with Hodgkin's lymphoma: a case study].

The case of a 17-year-old man with Hodgkin's lymphoma who presented with paraneoplastic sensory neuropathy is presented. The patient visited our hospital because of acute progression of dysesthesiae in the bilateral face and extremities. He also developed an ataxic gait due to decreased deep sensation. Post-contrast T1-weighted MRI showed enhancement of both trigeminal nerves and the cauda equina. Cerebrospinal fluid examination was unremarkable. Intravenous immunoglobulin therapy and subsequent steroid pulse therapy did not improve his symptoms. Laboratory data showed an elevated serum soluble interleukin-2 receptor level. His chest X-ray and CT showed enlarged lymph nodes in the mediastinum, and the histopathologic examination of a lymph node biopsy specimen showed classical Hodgkin's lymphoma. He was treated with chemotherapy. His symptoms of neuropathy improved promptly while the lymphoma was being successfully treated, and he was able to walk with a cane. The present case was characterized by paraneoplastic sensory neuropathy as the initial clinical feature in association with Hodgkin's lymphoma. It is necessary to consider a paraneoplastic neurological syndrome even in a young patient with acute/subacute sensory neuropathy. Paraneoplastic sensory neuropathy associated with Hodgkin's lymphoma could be expected to improve with oncotherapy, and examination of the malignancy and early treatment are important.

[Trends in the management of herpes simplex encephalitis].

Recent aspects of the diagnostic and therapeutic management of herpes simplex virus encephalitis (HSVE) are reviewed based on a clinical analysis of our adult patients. Detection of the HSV genome sequence in the cerebrospinal fluid (CSF) by the polymerase chain reaction (PCR) has been established as a gold standard diagnostic method at the acute stage of HSVE. However, several problems for the PCR in HSVE remain, as follows: the discrepancy in results based on differences of minimum detection sensitivities between the single PCR and nested PCR, and clinical pseudo-negative results which depend on the day of CSF sample collection after onset. Antiviral therapy is highly effective in reducing the mortality rate. However, only less than one-half of HSVE patients are able to return to normal. This finding indicates a need to develop a further improved therapeutic regimen. We recently reported an assessment of the efficacy of corticosteroid with aciclovir therapy for outcome in HSVE using multiple logistic regression analysis. A poor outcome was evident with older age, lower Glasgow Coma Scale scores at initiation of aciclovir, and no administration of corticosteroid. Combination therapy employing both aciclovir and corticosteroid is thus suggested to be useful for achieving a better outcome.

[A case of bilateral ophthalmoplegia caused by focal idiopathic hypertrophic pachymeningitis on the anterior cranial fossa].

A previously healthy 63-year-old man presented with a 2-weeks history of diplopia without headache. Neurological examination revealed total external ophthalmoplegia of the left eye and limitation of abduction of the right eye. Initial cranial MRI showed thickening and enhancement of the dura mater only on the anterior cranial fossa but unremarkable on the cavernous sinus. Idiopathic hypertrophic cranial pachymeningitis was diagnosed in the absence of demonstrable underlying infective, neoplastic, or systemic autoimmune disease by his clinical findings, laboratory tests and radiological examinations. Corticosteroid therapy was initiated with methylprednisolone (1,000 mg/day for 3 days), followed by oral prednisolone and tapering off. Eye movements improved with treatment and completely recovered within 4 weeks after starting administration, and cranial MRI at the 15 days after starting treatment showed improvement. We suggest that his ophthalmoplegia was caused by the inflammation of dura on the cavernous sinus beyond the thickening lesion of cranial MRI. In a case of bilateral ophthalmoplegia with or without headache, it is required to examine the dural thickening and enhancement on the anterior cranial fossa by cranial MRI.

Miller Fisher syndrome associated with influenza A infection.

A 36-year-old, previously healthy man presented with Miller Fisher syndrome (MFS) five days after he was diagnosed with an influenza A infection by a rapid antigen test. He had not received any recent vaccinations. He had no loss of consciousness. Bilateral ophthalmoplegia, blepharoptosis, areflexia, and ataxic gait were noted. One week after treatment with intravenous immunoglobulin, his ophthalmoplegia, blepharoptosis, and ataxic gait had gradually improved, and his deep tendon reflexes returned. Anti-GQ1b IgG antibodies were detected in his serum. There has been no previous report of postinfectious MFS following confirmed an influenza A infection in an adult.