Tomoko Muramatsu

Detection of preclinically latent hyperperfusion due to stroke-like episodes by arterial spin-labeling perfusion MRI in MELAS patients.

In stroke-like episodes (SEs) of patients with mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS), the detection of preclinically latent lesions is a challenge. We report regional cerebral hyperperfusion observed on arterial spin labeling (ASL) perfusion magnetic resonance imaging (MRI) in the preclinical phase more than 3 months before the clinical onset of SEs in 3 MELAS patients. These hyperperfused areas were not detected by conventional MRI in the preclinical phase and developed into acute lesions at the clinical onset of SEs, suggesting that ASL imaging has the potential for predicting the emergence of SEs.

Posterior reversible encephalopathy syndrome following measles vaccination.

Due to recent domestic measles outbreaks in Japan, the Japanese government has mandated measles vaccination at ages 14 and 17 since April 2008. Since then, the number of people receiving measles vaccination has increased in Japan. Measles vaccination may cause serious neurological complications including encephalopathy, although the incidence is very low. We report here an adult case of posterior reversible encephalopathy syndrome (PRES) and myeloradiculoneuropathy following measles vaccination. Brain MRI demonstrated typical findings of PRES, high intensity signals in the occipital lobes on FLAIR imaging, isointensity signals on diffusion weighted imaging, with an increase in the apparent diffusion coefficient (ADC). Vasoconstriction mainly in the posterior cerebral arteries was detected by MRA. Physicians should keep in mind the possible occurrence of PRES and myeloradiculoneuropathy following measles vaccination.

Pathophysiological decrease in the regional cerebral blood flow in Hashimoto's encephalopathy : a multiple-case SPECT study

Background: The aim of this study was to evaluate the changes in regional cerebral blood flow (rCBF) in multiple cases of Hashimotos encephalopathy (HE). Methods: Seven untreated patients with HE and 10 age-matched healthy controls underwent brain single photon emission computed tomography (SPECT) with N-isopropyl-p-[123I]iodoamphetamine. All patients had anti-NH2-terminal of α-enolase autoantibodies (Abs), which served as a useful diagnostic marker for HE, in addition to anti-thyroid Abs in their sera and responded to corticosteroid therapy. The obtained SPECT images were compared between the patients and the controls using 3D-SSP analysis. Results: The rCBF of all patients with HE was significantly decreased in the bilateral anterior cingulate areas and left prefrontal cortex compared with the controls (p < 0.05). Focusing on the HE patients with acute neuropsychiatric symptoms (n = 5) such as consciousness disturbance and/or psychosis, the decreased rCBF in these areas was more significant, and the rCBF in the right frontal cortex was also decreased. Conclusion: Statistical analysis of these multiple-case SPECT images revealed the regions of decreased CBF associated with clinical symptoms, especially acute neuropsychiatric symptoms, in HE patients. This study shed light on the pathophysiological decrease in rCBF observed in HE.

Periodic synchronous dischargeを呈しCreutzfeldt-Jakob病との鑑別を要した橋本脳症の1例

Here, we report a case of Hashimotos encephalopathy (HE) mimicking Creutzfeldt-Jakob disease (CJD). A 57-year-old man was admitted to our hospital for status epilepticus. He had gradually presented personality change over the last two years. On admission, he was in state of akinetic mutism. He exhibited seizures on the left side of his body, including the face, and intermittent myoclonic movement. Routine laboratory tests showed no abnormalities, including thyroid functions. An EEG study showed typical periodic synchronous discharge (PSD). Brain MRI showed high-intensity areas in the bilateral frontal cortex, thalamus, and right insula on diffusion-weighted imaging (DWI). So, initially, sporadic CJD was suspected. However, there were no abnormalities in the caudate or putamen on MRI. Anti-TG and anti-TPO antibodies, as well as anti-NAE antibody were all positive. He was administered methylpredonisolone pulse therapy. Subsequently, his consciousness levels and EEG and MRI findings markedly improved. So, he was finally diagnosed with HE. HE should be considered in patients with PSD on EEG, even if the patients have typical MRI abnormalities of CJD. Anti-thyroid antibodies should be examined in such patients.

[A long-term follow up of right ophthalmoplegia and severe right middle cerebral artery stenosis with right herpes zoster ophthalmics: a case report].

Here we report a case of a 68-year-old man with severe stenosis of the right middle cerebral artery (MCA) following herpes zoster ophthalmicus. He presented with right-sided ptosis and ophthalmoplegia 2 months after herpes zoster ophthalmicus. Cerebrospinal fluid (CSF) analysis revealed monocytosis, increased protein levels, and positivity for herpes zoster virus immunoglobulin M (IgM). Brain magnetic resonance imaging (MRI) revealed a small asymptomatic infarct in the right basal ganglia and severe stenosis of the right MCA (M1 segment). One month later, he presented with muscle weakness of the fingers of the left hand. Repeat CSF analysis revealed similar abnormalities to the previous analysis, and MRI showed a new small infarct in the right-sided motor area corresponding to the left fingers. He was treated with acyclovir (750 mg/day), prednisolone (1 mg/kg/day), and aspirin (100 mg/day). O2-gas positron emission tomography (PET) revealed decreased cerebral blood flow (CBF) after acetazolamide injection and normal cerebral vascular reactivity (CVR). He was on continuous treatment with prednisolone and aspirin for 1 year. The muscle weakness of the fingers of the left hand and right-sided ophthalmoplegia improved, and magnetic resonance angiography revealed considerable decrease in the stenosis of the right middle cerebral artery. CBF before and after acetazolamide injection and CVR on O2-gas PET also normalized. These results suggested that long-term treatment may prevent subsequent infarcts following herpes zoster ophthalmics.

[Simultanagnosia and scene agnosia induced by right posterior cerebral artery infarction: a case report].

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.

Delusions of theft in patients with dementia

Background: Laminaria japonica , an edible brown seaweed, has long been utilized since ancient times as an important food resource to promote maternal health in Pacific and Asian countries. Due to its high contents of dietary fiber, minerals, carbohydrates, and protein, L. japonica has recently attracted much attention for its bioactive materials and nutraceutical qualities. Methods: The present study examined the effects of desalted Laminaria japonica (D LJ) on learning and memory function and cholinergic neuron activity in trimethyltin (TMT) induced memory deficits rats.After TMT injection (8mg/kg, i.p.), rats were administered with saline or D LJ (10 and 50mg/kg, p.o.) daily for 21 days. The cognitive improving effects of D LJ on TMT-induced amnesic rats were investigated by assessing the Morris water maze test and by performing choline acetyltransferase (ChAT) immunohistochemistry.Results: In theMorris water maze, TMT treated group produced impairment in escape latency to find the platform. However, DLJ treated groups were significantly recovered the impairment of memory compared to the control group (on the 2nd and 4th day (P <0.05)). Consistent with behavioral data, DLJ group was significantly increased ChAT-ir neurons in the midial striatum compared to the control group (P <0.01). Conclusions: In conclusion, administration of D LJ improved spatial learning and memory. These results suggest that D LJ may be useful for the cognitive improvement via regulation of cholinergic marker enzyme activity.