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Akihisa Matsumoto

Publications and source records attributed to Akihisa Matsumoto.

13 recordsLinked to original sources

Reversible posterior leukoencephalopathy syndrome in p-ANCA-associated vasculitis.

The perinuclear antineutrophilic cytoplasmic antibody (p-ANCA) is closely associated with rapidly progressing glomerulonephritis, microscopic polyangiitis, and allergic granulomatous angiitis. While mononeuropathy due to vasculitis is a well-known neurological manifestation of these conditions, manifestations involving the central nervous system (CNS) have rarely been reported. Our patient presented the very characteristic CNS lesion of reversible posterior leukoencephalopathy syndrome (RPLS) which has often been associated with hypertension, eclampsia, cyclosporine neurotoxicity, and other diseases. The patient also developed the recently established disease entity, Takotsubo cardiomyopathy.

Aged↗

[A case of adult onset subacute sclerosing panencephalitis; sequential clinico-radiological findings over two years].

We present a very rare case of adult onset subacute sclerosing panencephalitis (SSPE), and explain the characteristic sequential clinico-radiological findings. The patient, a twenty three-year-old man, had noticed unsteadiness in walking about two months previously. Although inosine pranobex and intrathecal interferon were administered, symptoms worsened insidiously and he became bedridden after four months. The levels of serum and CSF anti-measles IgG antibodies have not changed. Initially, supratentorial cortical atrophy was observed, especially of the left temporal lobe, but there were no other MRI signal alterations at the time. After three months, the supratentorial cortices produced low-signal intensities in T1-weighted images and in T2-weighted MRI, the cortical margin was very unclear and white matter signal intensities had become higher. Furthermore, cortices became thinner and ventricular size increased, especially for the lateral and third ventricles. SPECT examinations showed a marked reduction in cerebral blood flow and the perfusion deficits observed seemed to be closely correlated with the abnormal MRI signal patterns. Pathological examinations of biopsy samples revealed infiltration of inflammatory cells around the small vessels. As for immunohistochemical findings, CD68 positive cells were frequently observed, and this result implied the activation of microglia. Further studies are necessary to elucidate the pathogenic mechanisms of SSPE.

Adult↗

Clinical analysis of longstanding subacute myelo-optico-neuropathy: sequelae of clioquinol at 32 years after its ban.

One thousand and thirty-one longstanding patients with subacute myelo-optico-neuropathy (SMON; 275 males, 756 females; mean age +/- S.D., 72.9 +/- 9.6 years; age at onset 37.6 +/- 9.8 years; duration of illness 35.3 +/- 4.0 years) were examined in 2002, 32 years after banning of clioquinol. At onset, 66.7% of patients were unable to walk, and 4.7% complete blindness. At present time, about 41% of patients were still difficult to walk independently, including 15.8% of completely loss of locomotion. One point six percent of patients were in complete blindness and 5.8% had severe visual impairment. The majority (95.6 - 97.7%) of patients exhibited sensory disturbances including superficial and vibratory sensations and dysesthesia. Dysautonomia was observed as leg hypothermia in 79.8%, urinary incontinence in 60.7%, and bowel disturbance in 95.3%. As complication, high incidence was revealed with cataract (56.2%), hypertension (40.2%), vertebral disease (35.5%), and limb articular disease (31.5%). These results indicate the serious sequelae of clioquinol intoxication, SMON.

Aged↗

Hodgkin's disease-related central nervous system angiopathy presenting as reversible posterior leukoencephalopathy.

Reversible posterior leukoencephalopathy syndrome (RPLS) is a clinical syndrome characterized by headache, conscious disturbance, seizure, and cortical visual loss with neuroimaging finding of edema in the posterior regions of the brain, with a reversible course (1). The precise pathomechanism of RPLS is not understood. However, association with uncontrolled hypertension, renal failure, eclampsia, or immunosuppressive agents has been implicated (1). We describe herein a case of Hodgkin's disease (HD)-related central nervous system (CNS) angiitis with neuroimaging finding suggestive of RPLS. The pathophysiology of RPLS in cases with CNS angiitis is discussed.

Adult↗

Primary CD30/Ki-1 positive anaplastic large cell lymphoma of the central nervous system occurring in a patient with a seventeen-year history of essential thrombocythemia.

We present a rare case of central nervous system lymphoma that occurred in a patient who had essential thrombocythemia for 17 years. MRI examinations disclosed multiple ring-enhanced lesions that had shown bilateral spreading in the different period. Pathological examinations confirmed CD30/Ki-1-positive ALK negative anaplastic large cell lymphoma. The possible pathogenic mechanisms of this disease are discussed.

Brain Neoplasms↗

[Two cases of hypertrophic cranial pachymeningitis associated with infection in the external auditory canal and paranasal sinus].

We here present two cases of hypertrophic cranial pachymeningitis exhibiting unique multiple cranial neuropathies, both of which were associated with otic and paranasal infections. Case 1: A 76-year-old woman developed headache after undergoing surgical dilatation of the external auditory canal, with subsequent development of a bacterial infection. Neurological examination reveled only bilateral hearing disturbance. MRI and CT scans demonstrated thickening of the dura mater and inflammatory granulation around the left cerebellar tentorium. Based on a diagnosis of hypertrophic pachymeningitis associated with previous infection, antibiotics were administered, followed by oral prednisolone therapy. This treatment relieved the headache and improved the MRI findings. However, 2 months later, the headache became worse and impaired movement of the soft palate, atrophy of the left side of the tongue, and atrophy of the sternocleidomastoideus muscle were noted. MRI revealed aggravated inflammatory changes around the left cerebellar tentorium and their expansion into the jugular foramen. Occlusive changes in the transverse and sigmoid sinuses were also seen. Case 2: A 78-year-old man developed bilateral visual loss, right frontal headache, and bilateral restriction of eye movement. He had been treated for phemphigus with prednisolone and azathioprine. MRI showed hypertrophic dura mater spreading continuously from the frontal base and ethmoid and frontal sinuses to the falx and right frontal lobe. Since Pseudomonas aeruginosa was cultivated in biopsy specimens from the dura mater, antibiotic agents were administered. The clinical symptoms resolved and MRI findings gradually improved.

Aged↗

Elbow flexion response as another primitive reflex.

In daily clinical practice we noticed that patients with intellectual impairment spontaneously flex the elbow within a few seconds of the forearm being manipulated during routine examination of spasticity of the muscles in the upper extremities. We termed this phenomenon elbow flexion response (EFR), and prospectively studied it in 229 patients who underwent in-hospital rehabilitation following brain damage. Evaluation of each patient included EFR, patient profile, ability to communicate, scores on three parameters from various intelligence tests, scores on seven parameters testing primitive reflexes, and scores on three parameters describing personality. We investigated for relationships among these parameters. Consequently, although EFR rarely have a statistical association with the varied profiles of patients, patients with bilateral lesion or bilateral paresis demonstrated significantly more marked EFR than those with unilateral lesion or unilateral paresis. Patients with involvement of the frontal lobe showed significantly more marked EFR than those without damage in this area. Elbow flexion responses occurred significantly more frequently in relation with lower scores on intelligence and occurred with significantly higher frequency in conjunction with the more marked appearance of conventional primitive reflexes. Therefore, we conclude that EFR have a strong association with intelligence and with the existence of frontal lobe lesion, and their mode of clinical presentation parallels that of primitive reflexes particularly that of the grasp reflex. We propose that EFR could be referred as a variation of the grasp reflex occurring in the more proximal or axial part of the body.

Brain Injuries↗

[A case of Ramsay Hunt syndrome initiated with hoarseness and dysphagia: consideration on spreading mechanisms of cranial neuropathy].

A 85-year-old woman was admitted to our hospital because of progressive hoarseness and dysphagia of two days' duration. Neurological examination on admission revealed right pharyngeal and vocal cord palsies. After admission, gradual swelling of her right ear was noted, and on day 6, vesicular eruptions in her right geniculate zone, the right VII and the VIIIth cranial nerve palsies were added. On the basis of these findings, she was diagnosed as Ramsay Hunt syndrome. Varicella zoster virus (VZV) infection was confirmed by the elevation of serum anti-VZV-antibody titer, and detection of VZV DNA from cerebrospinal fluid. Ramsay Hunt syndrome associated with multiple cranial neuropathy is not frequently reported. Reviewing Japanese literatures, we found that the IX and the Xth cranial nerves were most frequently affected, and the half of these cases were initiated with cranial neuropathy other than the VIIth. Additionally, spreading mechanisms of cranial neuropathy, and the early diagnostic problems of these conditions were discussed.

Aged↗