[A case of Alice in Wonderland syndrome after Epstein-Barr virus (EBV) encephalitis: a mimicry of focal epileptic seizure].

A 30-year-old man who received infliximab for treatment of Crohn's disease developed Epstein-Barr virus (EBV) encephalitis, which responded well to therapy; however, he had left lower visual field loss following treatment. The patient noticed peculiar symptoms 9 months after recovery from encephalitis; objects in his view appeared smaller or larger than their actual size (micropsia/macropsia). Moreover, it appeared that objects outside moved faster or slower than their actual speed of movements and moving objects appeared as a series of many consecutive snap shots. His vision was blurred, and he had visual difficulties and a sensation that his body was floating. These symptoms mainly appeared following fatigue and persisted over approximately 10 years. Based on cerebrospinal fluid analysis, brain MRI, N-isopropyl-p-123I-iodoamphetamine with single photon emission computed tomography, fluorodeoxyglucose positron emission tomography, and electroencephalography, we excluded both recurrent encephalitis and focal epileptic seizures. By taking all symptoms and other evaluation findings into account, the patient most likely suffered from "Alice in Wonderland syndrome" which is primarily associated with cortical dysfunction in the right temporo-parieto-occipital area as the consequence of previous acute EBV encephalitis.

Montreal Cognitive Assessment score correlates with regional cerebral blood flow in post-stroke patients.

OBJECTIVE: Valid and reliable measures are needed to assess post-stroke cognitive impairment. The Montreal Cognitive Assessment (MoCA) has been considered a superior screening test to the Mini-Mental State Examination (MMSE) for patients with post-stroke cognitive impairment, particularly in executive function, which may be related to reduction in regional cerebral blood flow (rCBF). In this study, we determined whether MoCA and MMSE scores correlate with rCBF assessed with SPECT in the subacute phase after ischemic stroke. PATIENTS AND METHODS: We retrospectively enrolled 28 patients who were admitted to the Red Cross Otsu Hospital with acute cerebral infarction, which was confirmed by magnetic resonance imaging (MRI), if they underwent cognitive assessment (MoCA/MMSE) and 123I-IMP SPECT imaging within 3 weeks post-stroke during a study period of 5 months. Correlation analyses between rCBF and MoCA or MMSE scores were performed by statistical parametric mapping (SPM) and volume-of-interest (VOI) analyses. RESULTS: Total MoCA score correlated with the rCBF in the prefrontal cortex, cingulate cortex, caudate nucleus and thalamus by SPM analysis (uncorrected p < 0.001; cluster-level corrected p < 0.05). Among the subtest scores of MoCA, visuoexecutive function, attention, language and delayed recall scores were positively correlated with rCBF in the prefrontal cortex by VOI analysis (p < 0.05). However, total MMSE score did not correlate significantly with any of the rCBF measures. CONCLUSIONS: Post-stroke cognitive performance assessed with MoCA positively correlated with rCBF in brain regions mainly comprising the prefrontal-subcortical circuits. The findings of this hypothesis-generating study support the notion that MoCA is useful for assessing post-stroke cognitive status.

[Case of an elderly woman with dementia showing episodic involuntary movement of the tongue].

We report a 93-year-old woman with dementia who developed generalized convulsion and involuntary movement of her tongue. She could independently walk and eat meals until 8 months ago, however she turned into bedridden. When she was admitted to our emergency room due to status epilepticus, her tongue intermittently moved from the midline to the left. She could not eat or speak during this episodic tongue movement. MR imaging study revealed brain atrophy in the bilateral mesial temporal lobe, consistent with senile dementia of Alzheimer type. Despite her tongue movements seemingly developing to the generalized convulsion, EEG study did not indicate epileptiform discharges corresponding to this movement. Although antiepileptic drug therapy was effective, we needed polytherapy to control this movement. Paroxysmal tongue movements were previously reported in cases of epilepsy, brain tumor, and stroke, observed bilaterally in most cases. This episodic tongue movement would be rare in terms of the clear laterality. The etiology of this movement was presumed as focal seizure, palatal tremor, dyskinesia or others, but was undetermined. Episodic movements involving tongue decrease the quality of daily life especially in the elderly. Therefore, we should pay more attention to it and try to treat it earlier.

Bereitschaftspotential augmentation by neuro-feedback training in Parkinson's disease.

OBJECTIVE: Decreased early Bereitschaftspotential (BP) is one of the electrophysiological characteristics in patients with Parkinson's disease (PD). We examined whether PD patients could increase BP amplitude by means of neuro-feedback (NFB) training for their slow cortical potentials (SCPs). METHODS: We worked with 10 PD patients and 11 age-matched controls. BP was measured for self-paced button pressing by their right thumb. The subjects were instructed to make the introspective efforts to produce negative SCPs (negativation). The one-day session consisted of three trials, that is, the first BP, NFB training and the second BP, and each patient performed this routine for 2-4 days. Amplitudes of the first and second BPs were compared between the two groups that were divided depending on NFB performance. RESULTS: Good NFB performance had the tendency of larger early BP in the second BP recording than in the first one, whereas in the poor NFB performance the early BP was smaller in the second BP recording than in the first one in both patient and normal groups (p < 0.001). CONCLUSIONS: Good NFB performance of negativation could increase excitatory field potentials of pyramidal cells for the generation of early BP. SIGNIFICANCE: Voluntary regulation of SCPs could enhance BP in PD patients and in aged controls.

Negative motor seizure arising from the negative motor area: is it ictal apraxia?

PURPOSE: Seizure manifesting motor arrest, that is, negative motor seizure (NMS), is a rare epileptic condition in which only inability to conduct voluntary movements or praxis is produced, although consciousness is preserved. The negative motor area (NMA) seems to be responsible, but its generator mechanism has not yet been clarified. PATIENTS AND METHODS: Three patients manifesting NMS were investigated. Two patients (ages 33 and 17) with intractable frontal lobe epilepsy had subdural grid implantation for epilepsy surgery, and one (age 77) had scalp electroencephalography (EEG) monitoring. RESULTS: Ictal semiologies commonly observed, at least in the two patients, were found as follows; (1) indescribable or ill-localized aura, (2) repetitive involuntary vocalization, (3) inability to speak, (4) inability to move the extremities, and (5) subsequent evolution to positive motor seizures. Awareness and comprehension were preserved throughout the episode before generalized seizures. In two patients with epicortical EEG recording, ictal activity arose from the lateral NMA in one, and from the rostral supplementary motor area in the other. Cortical stimulation at NMA in one patient elicited symptoms identical to NMS. Another patient had scalp EEG and magnetic resonance imaging (MRI) abnormality, both suggesting the epileptogenic focus in the mesial frontal area. CONCLUSION: We showed that (1) NMS was a rare condition in patients with seizure focus in the frontal lobe, and (2) that the NMA was responsible for the symptoms. The documented state in the present study may reflect ictal apraxia, but it requires further investigation.

Improved cerebral function in mesial temporal lobe epilepsy after subtemporal amygdalohippocampectomy.

The functional changes that occur throughout the human brain after the selective removal of an epileptogenic lesion remain unclear. Subtemporal selective amygdalohippocampectomy (SAH) has been advocated as a minimally invasive surgical procedure for patients with medically intractable mesial temporal lobe epilepsy (MTLE). We evaluated the effects of subtemporal SAH on cerebral glucose metabolism and memory function in 15 patients with medically intractable MTLE with hippocampal sclerosis using [(18)F]-fluorodeoxyglucose PET (FDG-PET) and the Wechsler Memory Scale-Revised. The patients were evaluated before and 1-5 years (mean 2.6 years) after surgery. In patients with MTLE of the language-dominant hemisphere, the basal temporal language area was preserved by this surgical approach. Voxel-wise comparison of FDG-PET images was conducted using SPM5 to identify the brain regions showing postoperative changes in glucose metabolism (height threshold, P = 0.01 corrected for multiple comparisons; extent threshold, 100 voxels). During spatial normalization of the postoperative FDG-PET images, we used cost-function masking to minimize any inappropriate image distortion as a result of the abnormal signal within the surgically resected region. Postoperative glucose metabolism increased in extratemporal areas ipsilateral to the affected side, such as the dorsolateral prefrontal cortex, and the dorsomedial and ventromedial frontal cortices. Glucose metabolism also increased in the bilateral inferior parietal lobules and in the remaining temporal lobe regions remote from the resected mesial temporal region, such as the superior temporal gyrus and the temporal pole. By contrast, postoperative glucose metabolism decreased only in the mesial temporal area adjacent to the resected region. Postoperative verbal memory, delayed recall and attention/concentration scores were significantly better than preoperative scores regardless of the resected side. This study suggests that the selective removal of the epileptogenic region in MTLE using a subtemporal approach improved cerebral glucose metabolism in the areas receiving projections from the affected mesial temporal lobe. Cognitive improvement might result from a combination of good seizure control and minimizing the regions of the brain with postoperative functional impairment.