Michikazu Nakamura

Transient eye and nose pain as an initial symptom of pontine infarction

Headache is relatively common in Wallenberg’s lateral medullary syndrome.1,2⇓ For head pain associated with this syndrome, C.M. Fisher proposed two distinct components1: pain in the occipital, occipitofrontal, and nuchal areas attributed to ischemia of a vertebral artery and pain in the eye, nose, and cheek to ischemia in the nucleus of the descending root of the trigeminal nerve. Pontine infarction has rarely been reported to cause facial pain similar to that in Wallenberg’s syndrome.3-5⇓⇓ We report three patients with transient eye and nose pain ipsilateral to the pontine infarct. ### Case 1. A 42-year-old man suddenly developed sharp facial pain radiating from the right medial side of his eye to the nose while walking. The pain lasted for several minutes. He also had a peculiar feeling similar to epistaxis on the right side. Approximately 5 minutes later, he developed left-sided numbness …

Response to corticosteroid therapy in a patient with HTLV-I-associated motor neuron disease.

Human T-lymphotropic virus type I (HTLV-I)-associated myelopathy/tropical spastic paraparesis (HAM/TSP), or tropical spastic paraparesis (TSP), is an inflammatory disease involving mainly the thoracic spinal cord and is characterized by slowly progressive paraparesis, neurogenic bladder, and mild sensory changes. However, there have been reports of HTLV-I carriers presenting severe muscular atrophy, fasciculation, and pyramidal tract signs without obvious sensory abnormalities.1-4 The features of these patients mimic amyotrophic lateral sclerosis (ALS). This raises the question of whether these patients represent a clinical variant of HAM/TSP or a chance association of HTLV-I infection and ALS. To provide some insight into this issue, we performed a therapeutic trial with corticosteroid in an HTLV-I carrier with an ALS-like manifestation. Case report. A 48-year-old man was admitted to Tenri Hospital because of bilateral hand weakness. He was born and raised on HTLV-I-endemic Kyushu Island.5 At age 36, he developed weakness in all four limbs and sensory loss below both knees 1 week after a flu-like syndrome. He spontaneously recovered from this Guillain-Barre-like syndrome sometime …

Subacute dementia with necrotising encephalitis selectively involving the fornix and splenium: Retrograde development of Alzheimer's neurofibrillar tangles in the subiculum

An elderly woman developed subacute progressive dementia with predominant impairment in recent memory and CT findings indicating an enhancing lesion near the splenium of the corpus callosum. Autopsy four months after the onset of the symptoms revealed necrotising encephalitis selectively involving the bilateral fornices and the adjacent splenium. Degeneration was marked in the contiguous right fimbria hippocampi. Particularly numerous Alzheimers neurofibrillar tangles (NFTs) were present in the right subiculum, while they were scattered in Sommers sectors and parahippocampal gyri and were practically nonexistent elsewhere. Senile plaques were rare. Electron microscopy disclosed that the right subicular tangles were composed mostly of 15-nm straight tubules which were also frequently observed in the myelinated axons. Since the major projection fibers to the fornix originate in the subiculum, the distinctive pattern of NFT distribution might be derived from the retrograde neuronal changes secondary to the fornix-fimbria lesion. This case represents a rare form of amnesia-dementia confirmed anatomically to have been caused by fornix lesions.

A case of myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA)-associated hypertrophic pachymeningitis presenting with multiple cranial nerve palsies and diabetes insipidus.

A 61-year-old woman developed hearing difficulties and became thirsty after experiencing cold symptoms. A neurological examination revealed a loss of odor sensation, facial palsy, dysphasia, and dysarthria. Vocal cord palsy was observed during pharyngoscopy. Brain magnetic resonance imaging (MRI) showed a thickened pituitary stalk and swelling of the pituitary gland, but no high signal intensity regions were seen in the posterior portion of the pituitary gland. Gadolinium-enhanced MRI demonstrated a thickened dura mater over the anterior cranial fossa. A biopsy specimen of the thickened dura mater showed fibrosis, granulomatous inflammation, and necrotic foci. Blood tests detected myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA). The patients urine osmolarity was low even though she exhibited hypernatremia. We diagnosed her with hypertrophic pachymeningitis associated with MPO-ANCA and diabetes insipidus. The patient received two courses of 5-day high-dose intravenous methylprednisolone (1.0 g/day), and was subsequently administered oral prednisolone, which gradually relieved her symptoms. However, the patients symptoms recurred despite the high-dose prednisolone treatment. It was difficult to control the patients symptoms in this case with oral prednisolone monotherapy, but combined treatment with cyclosporine resulted in sustained remission. It is considered that patients with MPO-ANCA-positive hypertrophic pachymeningitis require combination therapy with prednisolone and immunosuppressive agents at an early stage.

Bifurcation of P9 far-field potentials induced by changes in the shoulder position.

Somatosensory evoked potentials to stimulation of the left median nerve were recorded from normal adults with reference to the right knee in the usual shoulder position and in an elevated shoulder position. A single peak of the P9 potentials in the former position bifurcated into two peaks in the latter position without changing the onset latency. This waveform change can be accounted for by changes in the resistance of the volume conductor around the nerve trunk.

A case of chronic inflammatory demyelinating polyradiculoneuropathy, showing radicular pain due to tuberous hypertrophy of the spinal roots and plexuses after 20 years interval without relapsing sensorimotor symptoms

A 40-year-old man visited our department because of chest and back pain. He had a history of diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP) 20 years ago. He received immunosuppressive therapy and had no relapses after that. On Admission, MRI showed tuberous hypertrophy of the spinal roots, intercostal nerves, and brachial and lumbar plexuses. The genetic analysis showed no mutations in any of Charcot-Marie-Tooth related genes. He was finally diagnosed with CIDP and administration of high dose intravenous methylprednisolone relieved his chest and back pain within a few days. We present a rare case of CIDP in which showed marked enlarged spinal roots in long clinical course and have a relapse with radicular pain without sensorimotor symptoms.

Pre-treatment blood pressure is the predictor for hemorrhagic infarction after intravenous rt-PA

Masaki Takata MD1, Yuuichi Masuda MD1, Yasuhiro Kawabata MD2, Shinsuke Nirengi PhD3, Tetsuya Tsukahara MD PhD2, Naoki Sakane MD PhD3 Department of Neurology1, Department of Neurosurgery2, and Division of Preventive Medicine, Clinical Research Institute3, National Hospital Organization, Kyoto Medical Center, Kyoto, Japan : Pre-treatment blood pressure is the predictor for hemorrhagic infarction after intravenous rt-PA

Effectivness of selective adenosine A2A receptor antagonist istradefylline on the motor symptoms of Parkinson’s disease correlates with the intracortical inhibition revealed by transcranial magnetic stimulation

1) Istradefylline, a selective antagonist to the adenosine A2A receptor on the GABAAergic medium spiny neurons in the striatum, reduces the duration of off-state in Parkinsons disease(PD). (2) Its effectiveness correlated with short-interval intracortical inhibition (SICI) studied by transcranial magnetic stimulation, which is a marker of cortical GABAAergic inhibition in the M1. (3) SICI may be an useful predictor of the effectiveness of istradefylline.

Predictors of Cardioembolic Stroke in Japanese Patients with Atrial Fibrillation in the Fushimi AF Registry

Background: Large-scale clinical trials have analyzed risk factors for any ischemic stroke in patients with atrial fibrillation (AF). However, the risk factors for cardioembolic stroke (CES), specifically, have not been reported. To clarify the risk factors for CES and clinically significant cardioembolic infarction, we examined the incidence of CES and larger infarct volume (IV) (> 30 mL) CES, employing the Fushimi AF Registry, a community-based prospective cohort of AF patients in the Fushimi ward, Kyoto, Japan. Methods: A total of 4,182 Fushimi AF patients were enrolled from March 2011 to December 2014. The risk factors for CES were evaluated using multivariate analysis. Results: Of 4,182 patients enrolled, 3,749 patients were observed for ≥1 year. During the follow-up period (mean duration, 979 ± 7.7 days), 91/3,749 patients experienced a CES (2.43%). Significant risk factors associated with CES were older age (odds ratio [OR], 1.31; 95% confidence interval [CI], 1.01–1.72; p = 0.046), low body weight (OR, 1.30; 95% CI, 1.03–1.65; p = 0.033), sustained AF (OR, 1.67; 95% CI, 1.05–2.71; p = 0.034), and previous stroke or transient ischemic attack (TIA) (OR, 1.94; 95% CI, 1.22–3.06; p = 0.004). Predictors of a large IV were chronic kidney disease (CKD) (OR, 2.08; 95% CI, 1.09–4.05; p = 0.027) and previous stroke/TIA (OR, 2.27; 95% CI, 1.19–4.24; p = 0.011). Conclusions: In this population-based cohort of Japanese patients with AF, in addition to previous stroke/TIA and older age, sustained AF and low body weight emerged as risk factors for CES, as opposed to any stroke, which may have a different risk profile. Patients with CKD or previous stroke/TIA who developed cardioembolic infarction exhibited more advanced severity. There is a need for direct oral anticoagulants that can be used safely in patients with comorbid AF and CKD.