Amnestic mild cognitive impairment (aMCI) has been considered to be a transitional state between healthy aging and very mild Alzheimer’s disease (AD). Most patients with aMCI convert to AD over time, but some of them remain stable as aMCI. In this study, 22 patients with aMCI underwent proton magnetic resonance spectroscopy (1H-MRS) of hippocampus and posterior cingulate cortex. Ten patients converted to AD had significantly lower N-acetylaspartate (NAA) concentrations in both hippocampi when compared to 12 patients remained stable to be aMCI. The mean NAA concentration of both hippocampi equal to or lower than 7.6 mmol/l predicted conversion to AD at 1.0 sensitivity and 1.0 specificity and the area under receiver operating curve (ROC) was 1.0. Absolute quantification of 1H-MRS of hippocampus seems to be a useful marker for predicting conversion to AD from patients with aMCI .

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We describe a case of 20-year-old woman with visual impairment in her left eye. Her left visual acuity was 0.07 and an ophthalmoscopic examination demonstrated bilateral intermediate uveitis (IU). A neurological examination on admission revealed lower nasal quadrantanopsia in her left eye and an exaggerated right patellar tendon reflex. A T₂-weighted MRI showed multiple high-intensity lesions in the bilateral periventricular region, corpus callosum, medulla. A short T₁ inversion recovery MRI also showed a swollen left retrobulbar optic nerve and posterior thoracic cord lesion at Th 9 level. The latter longitudinal length was approximately 20 mm. Laboratory investigation demonstrated no abnormalities including an anti-aquaporin-4 antibody. A cerebrospinal fluid examination revealed an increased IgG-index (1.21) with oligoclonal IgG babds. Initially, a diagnosis of retrobulbar optic neuritis with IU was made. She received subtenon corticosteroid injection with intravenous methylprednisolone pulse and oral prednisolone therapy. An immediate improvement of her visual symptoms and MRI abnormalities was observed. Approximately 1 year later, a new high-intensity lesion in the right internal capsule was present on a follow-up T₂-weighted brain MRI, established a diagnosis of multiple sclerosis (MS) based on the McDonald criteria in 2010. Previous reports in Japan demonstrated few cases of uveitis in patients with MS and this is the first report of MS with IU in Japan.

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A 68-year-old man was admitted to our hospital for rehabilitation of topographical disorientation. Brain magnetic resonance imaging revealed infarction in the right medial side of the occipital lobe. On neuropsychological testing, he scored low for the visual information-processing task; however, his overall cognitive function was retained. He could identify parts of the picture while describing the context picture of the Visual Perception Test for Agnosia but could not explain the contents of the entire picture, representing so-called simultanagnosia. Further, he could morphologically perceive both familiar and new scenes, but could not identify them, representing so-called scene agnosia. We report this case because simultanagnosia associated with a right occipital lobe lesion is rare.

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A 41-year-old right-handed woman presented abnormal behavior two weeks after suffering from headache and fever. Anti-glutamate receptor antibodies in the serum and cerebrospinal fluid were positive and we diagnosed anti-glutamate receptor antibody-related encephalopathy. The patient improved after administration of corticosteroid and was discharged without neurological deficit. After discharge, pure alexia and foreign accent syndrome-like language disturbance appeared consecutively. The serial fluorodeoxyglucose-positron emission tomography scans suggested that pure alexia and FAS-like language disturbance may have been caused by low function of the occipital lobes and the left frontal lobe, respectively. FAS has been linked to various lesions in the brain. The background mechanism may therefore be heterogeneous. On the other hand, patients with this syndrome recover spontaneously with our case. FAS may therefore be a temporal phenomenon resulting from imbalance in language processing rather than a specific deficit.

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A 49-year-old woman was admitted to our hospital with gradually progressive weakness of the limbs for about 20 days. She presented with weakness of the limbs, predominantly in the proximal portion, and slight dysesthesia of the limbs, predominantly in the distal portion. Repeated nerve conduction examination revealed axonopathy dominantly in the motor neurons. Therefore, we suspected her as having Guillain-Barré syndrome, and initiated intravenous administration of high-dose immunoglobulin. However, her symptoms progressed gradually and finally she found it difficult to walk. Her urine analysis simultaneously demonstrated albuminuria, and a kidney biopsy indicated focal segmental glomerulosclerosis. At that point, laboratory examination showed high levels of anti SS-A antibody and salivary gland biopsy revealed infiltration of a significant number of lymphocytes around the gland, which led to the diagnosis of Sjögren’s syndrome. We considered the etiology of the neural and renal dysfunction as due to the inflammatory mechanism associated with Sjögren’s syndrome. Therefore, we administered a second course of immunoglobulin therapy and steroid therapy, which included both pulse and oral administration. Her neurologic symptoms and albuminuria improved rapidly after steroid therapy. The present case indicates that both motor dominant neuropathy and focal segmental glomerulosclerosis can occur in patients with Sjögren’s syndrome.

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A 30-year-old woman was admitted to the first institution with subacutely progressive aphasia and depression. Despite of lacking conclusive evidence on magnetic resonance imaging, cerebrospinal fluid examination, or electroencephalogram, we tentatively diagnosed her disease as limbic encephalopathy due to its acute progression. High-dose methylprednisolone was started on admission. However, symptoms did not improve. To make matters worse, psychiatric symptoms, such as hallucinations and emotional incontinence, appeared on the same day. Additional treatment with plasma exchange and intravenous immunoglobulin administration was also ineffective. Therefore, we could not manage the patient in a general ward due to severe psychiatric symptoms. The patient was transferred to a psychiatric ward in the second institution. She received both psychiatric treatment and steroid therapy, including a second course of intravenous high-dose methylprednisolone, followed by long-term oral prednisolone. Her symptoms gradually improved. A final diagnosis of Hashimoto’s encephalopathy was made based on the patient’s clinical course and positive results for both serum anti-thyroid antibody and anti-NAE antibody. In our case, long-term oral steroid therapy under psychiatric treatment was effective for good outcome.

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We report two cases of young patients with lacunar stroke who demonstrated multiple microbleeds (MBs) a long period after cranial irradiation. Case 1: a 44-year-old man developed dysarthria and left hemiparesis from a day before admission. Magnetic resonance imaging showed an acute lacunar infract in the right corona radiata. Case 2: a 28-year-old woman was admitted due to dysarthria and right hemiparesis. An acute lacunar infract was detected in the left corona radiata. T₂* WI revealed multiple MBs in the region corresponding to the irradiation in both patients. Since these patients had no vascular risk factors except for smoking habit in the former one and dyslipidemia in the latter one. We speculated that lacunar stroke and MBs had resulted from past cranial irradiation. In addition to accelerated atherosclerosis of the large vessels, small vessel disease should be noted as an important long-term complication after cranial irradiation.

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A 34-year-old man developed right neck pain. Several hours later, he felt numbness of his extremities and presented at our hospital. He developed right hemiparesis and hypoesthesia of the right extremities. A few hours later, upbeat nystagmus and dysarthria appeared along with a sensory disturbance that spread to all extremities, and right hemiparesis progressed to tetraplegia. Brain MR diffusion-weighted images revealed a high-intensity lesion in the bilateral medial medulla oblongata and we diagnosed this bilateral medial medullary infarction. Three dimentional CT angiography revealed dissection of the right VA. We administered intravenous argatroban, edaravone, glycerin and oral clopidogrel. He was assessed as having modified Rankin scale 4 and was transferred to another hospital for rehabilitation on day 30. When the medial medulla oblongata is supplied by the unilateral VA, a unilateral VA dissection can cause bilateral medial medullary infarction.

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A 45-year-old female with a history of dry eyes presented with chronic progressive disturbance of her right finger extension, bilateral foot drops, and dysesthesia in the left lower leg. On admission, neurological examination revealed decreased tendon reflex in the right upper limb and bilateral lower limbs, and dysesthesia in the distal outer portions of the bilateral lower legs. Her vibration sensation was moderately diminished in both lower legs. Weakness ranging from moderate to severe was predominantly found in the muscles innervated by the radial (r > l), medial (r > l), ulnar (r > l), deep peroneal (r ≒ l), tibial (r < l), and medial planter nerves (r < l). Nerve conduction study showed asymmetrical axonal neuropathy, while chronic neurogenic changes were observed on needle electromyography. Cerebrospinal fluid analysis results were unremarkable, except for an elevated IgG index (1.53) and the presence of oligoclonal IgG bands. Seropositive anti-SS-A antibody and histological findings of the biopsied minor salivary gland, as well as the lack of other connective tissue disorders, indicated primary Sjögren syndrome (SjS). A left sural nerve biopsy showed inhomogeneous reductions in the myelinated fibers within fascicules, mild infiltration of CD8-positive T lymphocytes around small vessels, and no fibrinoid necrosis in the arteries. From these findings, the diagnosis of motor-dominant multiple mononeuritis associated with primary SjS was made. Therapy that comprised a single course of intravenous (IV) methylprednisolone (1 g for 3 days), followed by oral prednisolone (60 mg/day) with gradual tapering, resulted in no amelioration of her symptoms. She then received IV immunoglobulin (0.4 mg/kg/day for 5 days), which resulted in moderate improvement in the strength of several muscles and a reduction of CSF IgG index (0.89). A wide variety of peripheral nerve complications are documented in primary SjS. However, the present case is unique in the symptoms of chronic progressive, motor-dominant, multiple mononeuritic phenotype.

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The patient is a 68-year-old Japanese woman. She was admitted to our hospital due to continuous hiccups and vomiting episodes for more than one week. On examinations, muscle strength in her right lower limb was slightly decreased, and pyramidal tract signs were positive bilaterally. The fluid attenuated inversion recovery imaging of the brain showed lesions in the dorsal and lateral medulla. Serum anti-aquaporin 4 antibody was positive. We then diagnosed the patient with neuromyelitis optica spectrum disorder (NMOSD). Severe orthostatic hypotension (OH) was determined. While hiccups and vomiting improved gradually, OH lasted for more than three weeks. OH improved after administration of intravenous methylpredonisolone-pulse therapy. In this case, the lesion in the dorsal medulla might be responsible for OH. We considered that OH might be one of the symptoms of NMOSD.

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[in Japanese]

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[in Japanese]

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