Cortical spreading depression (CSD) is believed to be a phenomenon underlying migraine auras. The mutations of hemiplegic migraine genes are demonstrated to cause a reduction of CSD threshold. Consistently, tonabersat, which was developed for its ability to inhibit CSD, showed a preventive effect on attacks of migraine with aura. Besides, CSD has also been reported to activate the trigemino-vascular system, which subsequently causes migraine headache. The transient receptor potential cation channel, subfamily V, member 1 (TRPV1) receptor is known as one of the nociceptive receptors, and exists in the dura mater and the trigeminal ganglion. We demonstrated that the dural TRPV1 receptor conducts pain sensation to the trigeminal nucleus caudalis via the trigeminal ganglion, which implies possible contribution of the TRPV1 receptor to migraine headache. Also our recent data have raised the possibility that the TRPV1 receptor may play a pivotal role for the chronification of migraine. Furthermore, the TRPV1 receptor regulates the release of calcitonin gene-related peptide (CGRP). CGRP has been recognized to be associated with migraine because of its potent effect for dilation of intracranial and extracranial blood vessels. Some newly developed CGRP receptor antagonists have revealed the efficaciousness for acute migraine attacks. The present review discusses the relevance of recent advance of basic migraine research to future migraine treatment.

View full abstract

We reported two women (78 and 85 years of age) with dropped head syndrome caused by hypokalemic myopathy restricted to the posterior cervical muscles. Both presented with relatively rapid onset of severe neck extensor weakness. Needle EMG demonstrated myogenic changes in the cervical paraspinal muscles and there were high intensity signals in the posterior cervical muscles on the neck MRI. Dropped head syndrome resolved in both patients as potassium normalized. One of the patients relapsed 11 months later with recurrent hypokalemia, but recovered rapidly with supplementation of potassium. Focal myopathy localized in the posterior cervical muscles due to hypokalemia should be considered as one of the possible causes of dropped head syndrome.

View full abstract

Herein, we present a case of amaurosis fugax in a 76-year-old female with high blood pressure, dyslipidemia, and chronic atrial fibrillation who frequently suffered from transient visual impairment of the left eye. Color Doppler imaging of the flow profile in the left ophthalmic artery and the left common carotid artery revealed stenosis in the proximal portion of the left common carotid artery. Aortography revealed severe stenosis of the left common carotid artery at its orifice. The left external carotid artery was not observed as no collateral circulation from the left external carotid artery to the left ophthalmic artery was present. The presence of severe stenosis of the left common carotid artery at its orifice was considered to have caused the amaurosis fugax of the left eye, due to a reduction in the blood supply from both the left external carotid artery and the left internal carotid artery. Percutaneous transluminal angioplasty with stenting was successfully performed to dilate the stenosis at the orifice of the left common carotid artery with the aim of restoring visual function by improving the blood supply to the left ophthalmic artery. After stenting, angiography of the left common carotid artery showed improved blood supply in the left ophthalmic artery. The patient was followed up for 2 years after CAS, and no recurrence of amaurosis fugax or rubeosis iridis were observed. In this case, color Doppler imaging was useful for diagnosing and evaluating flow dynamics.

View full abstract

We report on a Japanese 13-year-old male without a family history of muscle disease admitted to our hospital due to an elevated serum creatine kinase. From the age of 3 he was complaining of muscle stiffness during and after exercise. At the age of 7 he experienced muscle stiffness and weakness during long-distance running, which would continue till the next day, disappearing only after resting for a day. Upon examination, we noted that repeated eyelid contractions induced myotonia that increased in the cold. Electromyography revealed myotonic discharge in the tongue muscle. Genetic analysis revealed a mutation of Nav1.4, M1592V. Although this mutation had originally been reported in families with Hyperkalemic periodic paralysis (Hyper PP), we diagnosed as paramyotonia congenita due to the symptoms of exercise and cold-induced myotonia without an attack of generalized weakness. This case suggest that sodium channelopathy is very rare, but should be considered in the differential diagnosis of an elevation of serum creatine kinase even if coexisting myotonia is only mild.

View full abstract

A 77-year-old woman with bilateral horizontal gaze palsy, right hemifacial weakness and incomplete quadriplegia was transferred to our hospital. Brain magnetic resonance imaging on the first day revealed a slit-like signal deficit of the basilar artery and an abnormal signal area at the dorsal midline portion of the lower pons. Quadriplegia fluctuated in several days after admission, then disappeared finally. In spite of the recovery of quadriplegia, bilateral facial weakness appeared on Day 14 after the onset. Concerning the impairment of extraocular movements, bilateral adduction restored gradually followed by improvement of the right abduction. The clinical course suggested the involvement of bilateral medial longitudinal fasciculus (MLF) and abducens nuclei (or fibers) as the etiology of gaze palsy. Although bilateral MLF sign recovered within 3 weeks, and the abductor palsy of both eyes was persisted in mild degree. As imaging analysis did not always show the causative lesion, which correlated with the rapidly alternating signs in the patient, and careful neurological observation was therefore useful in the management of patients with brainstem dysfunction.

View full abstract

We report the case of a 73-year-old woman with progressive gait disturbance for several months and an impaired standing ability. Her symptoms were lower-limb-dominant spastic tetraplegia with normal muscle tonus, hyperreflexia with positive Babinski sign, hypothermesthesia and hypalgesia in bilateral lower limbs, and dysesthesia in the fingers of both hands. Magnetic resonance (MR) images revealed a long lesion extending to nearly the entire spinal cord, and chest computed tomography (CT) revealed diffuse multiple small nodular lesions in the lower parts of the right and left lungs. High titers of human T-cell lymphotropic virus (HTLV)-1 antibodies in the serum and CSF, large amount of HTLV-1 provirus and neopterin in the CSF, and lymphocytic infiltration around bronchioles in pulmonary biopsy specimens indicated the presence of HTLV-1-associated myelopathy (HAM) with HTLV-1-associated bronchiolo-alveolar disorder (HABA). Her neurologic symptoms and the lesions in the spinal cord and lungs rapidly improved after corticosteroid pulse therapy. Rapid progressive HAM should be considered as a differential diagnosis of subacute spastic paraparesis with long spinal cord lesions. Further, careful investigation to detect pulmonary lesions, which are reported to be asymptomatic in most cases, is useful to confirm a diagnosis of HAM.

View full abstract

We report a case of limbic encephalitis repeated aphasic status epilepticus with periodic lateralized epileptiform discharges (PLEDs) . A 51-year-old man developed convulsions, psychiatric symptoms such as anxiety, phobia and ease of anger, and Wernicke's aphasia. Analysis of the cerebrospinal fluid (CSF) showed increase of leukocyte count (148/μl, mononuclear cells). Brain magnetic resonance imaging (MRI) showed hyperintensity lesions in the left medial temporal area and basal frontal area on T₂-weighted and fluid-attenuated inversion recovery (FLAIR) images. The electroencephalography (EEG) showed PLEDs over the left hemisphere, occurring at intervals of 0.5-1Hz. Although his limbic symptoms improved, Wernicke's aphasia occurred perioiodically with PLEDs appearance. After the administration of antiepileptic drugs, his language performance improved, and PLEDs were completely disappeared. We diagnosed him limbic encephalitis with non-convulsive repeated aphasic status epilepticus with periodic lateralized epileptiform discharges. Aphasic status epilepticus should be considered in the patients with limbic encephalitis, and careful evaluation of aphasia and EEG should be necessary to diagnose of aphasic status epilepticus.

View full abstract

Chronic inflammatory demyelinating polyneuropathy (CIDP) is classified into typical type that is characterized by proximal weakness and atypical type that include demyelinating acquired distal symmetric (DADS) type. DADS type CIDP is characterized by slowly progressive clinical course, motor and sensory involvement dominant in the leg and resistance to therapy. We report the case of a 72 year-old man with DADS type CIDP, who have dysesthesia and weakness dominant in distal of limbs. Disease progression was resistant to therapy for prednisolone and intravenous immunoglobulin, but slowly progressive for 4 years. Subacute exacerbation that was accompanied by proximal weakness was occurred at 4 years later from onset. Nerve conduction study revealed markedly prolonged distal latencies and slowing of conduction velocities. Double filtration plasmapheresis improved symptom, after oral prednisolone kept remission. This case had characteristic of DADS type CIDP at the onset, but changed into typical CIDP also for the therapy in the clinical course. This case indicate the multiplicity of CIDP.

View full abstract

A 77-year-old woman with cognitive impairment and multifocal progressive lesions on brain MRI was admitted to our hospital. Analysis of blood and cerebrospinal fluid showed no evidence of infection, autoimmune disease, or metabolic abnormalities. Histological examination of biopsied tissue from a lesion in the right frontal lobe revealed an abnormally increased glial cell density with enlarged nuclei and a high MIB-1 index. These pathological findings coupled with her progressive clinical history indicated a diagnosis of gliomatosis cerebri. General characteristics of gliomatosis cerebri include diffuse infiltrative lesions in neuroimaging with or without mass effect. However, the present case showed unusual multifocal manifestations in brain MRI. Therefore, histopathological examination must be taken into account for a proper diagnosis.

View full abstract