[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.

SARS-CoV-2-related Progressive Brain White Matter Lesion Associated with an Increased Cerebrospinal Fluid Level of IL-6.

We herein report a 49-year-old man with a fever, diagnosed with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. After two weeks of hospitalization, he suddenly mentioned visual field impairment. Computed tomography and magnetic resonance imaging revealed white matter damage and vasogenic edema. Cerebrospinal fluid showed increased levels of interleukin (IL)-6. His symptoms and white matter lesion deteriorated. After treatment with intravenous methylprednisolone therapy and plasmapheresis, his symptoms and white matter lesion improved gradually. We suspect that our patient was affected by a secondary hyperinflammatory syndrome related to cytokines, alone or in combination with direct viral injury through endothelial cell damage. The IL-6 levels were elevated only in the cerebrospinal fluid, suggesting focal central nervous system inflammation.

A case of human immunodeficiency virus infection with cerebellar ataxia that suggested by an association with autoimmunity.

We report a case of human immunodeficiency virus (HIV) infection that showed subacute progressive cerebellar ataxia without HIV encephalopathy or other encephalopathies, including progressive multifocal leukoencephalopathy or encephalitis of other human herpes virus (HHV) infections. A 43-year-old man exhibited unsteady gait. Neurological examination disclosed ataxia of the trunk and lower extremities. Personality change and dementia were absent. Magnetic resonance imaging did not reveal any abnormal finding, including of the cerebellum. The serum HIV-1-RNA was 1.2 × 10(5) copies/ml, and the absolute CD4 lymphocyte count was 141 cells/ml. Remarkably, the serum anti-Yo antibody, as an anti-cerebellar antibody of paraneoplastic syndrome, and anti-gliadin antibody, associated with celiac disease or gluten ataxia, were positive. The cerebrospinal fluid (CSF) immunoglobulin G index was 1.2 (< 0.8), and oligoclonal bands were present. PCR of the CSF was negative for HIV, JC virus, other HHVs, and mycosis. Previous reports presented HIV-infected patients with concurrent autoimmune diseases such as systemic lupus erythematosus, anti-phospholipid syndrome, autoimmune thrombocytopenia, vasculitis, polymyositis and dermatomyositis, sarcoidosis, Graves' disease, and hepatic diseases. These might have been present in patients with a CD4 T lymphocyte count of more than 200 cells/ml. On the other hand, paraneoplastic syndrome, gluten ataxia, cerebellar ataxia associated with anti-glutamic acid decarboxylase antibody, and Hashimoto's encephalopathy might manifest as autoimmune cerebellar ataxia. As regards the association of HIV infection and autoimmune cerebellar ataxia, a previous report suggested that anti-gliadin antibody was detected in about 30% of HIV-infected children, though there is no reference to an association with cerebellar ataxia. Moreover, to our knowledge, detection of anti-Yo antibody in an HIV-infected patient with cerebellar ataxia has not been reported. These findings suggest that, although it is extremely rare, clinicians need to consider HIV infection in a patient exhibiting autoimmune cerebellar ataxia.

Network specific change in white matter integrity in mesial temporal lobe epilepsy.

OBJECTIVES: To identify the specific change of white matter integrity that occurs in the brain network related to epileptic activity in patients with mesial temporal lobe epilepsy (MTLE). METHODS: We recruited 18 patients with MTLE and 18 healthy subjects. In MTLE patients, the remote functional-deficit zone was delineated using fluorodeoxyglucose positron emission tomography as an extratemporal region showing glucose hypometabolism. Using diffusion magnetic resonance imaging tractography, we defined a seizure propagation tract (PT) as a white matter pathway that connects the focus with a remote functional deficit zone. We also used the corticospinal tract (CST) and inferior longitudinal fasciculus (ILF) as control tracts in the hemisphere ipsilateral to the focus. Fractional anisotropy (FA), mean diffusivity (MD), and volume of the tracts were compared among PT, CST, and ILF. RESULTS: Tractographic analysis identified the uncinate fasciculus, arcuate fasciculus, and fornix as PTs. A decrease in FA was found in MTLE patients compared with healthy subjects in all tracts, but PTs showed a more significant decrease in FA than did the two control tracts. Although the change in MD was also found in MTLE patients compared with healthy controls, a tract-specific change was not observed. Although white-matter damage was observed in all candidate tracts examined, the integrity of white matter was most significantly decreased in PTs in MTLE. CONCLUSION: The change in white matter integrity occurs specifically in the pathways that connect the focus and remote functional deficit zones in patients with MTLE, i.e., the pathways that are assume to be associated with seizure propagation.

Increased clinical anticipation with maternal transmission in benign adult familial myoclonus epilepsy in Japan.

We recently reported clinical anticipation in Japanese families with benign adult familial myoclonus epilepsy (BAFME). However, it remains unknown whether clinical anticipation is predominantly associated with paternal or maternal transmission. We investigated the relationship between gender of the transmitting parent and clinical anticipation in nine BAFME families. Clinical anticipation regarding either cortical tremor or generalised seizures was observed in all 12 parent/child pairs (8 mother/child pairs and 4 father/child pairs). Moreover, a higher degree of clinical anticipation was associated with maternal transmission than with paternal transmission (p=0.03). Although a causative gene for BAFME still remains unknown, our finding suggests that BAFME and diseases with unstable expanding repeats, including those in non-coding regions, might share a similar molecular mechanism because such diseases often show clinical anticipation with maternal transmission.

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.

[A case of myasthenia gravis presenting solely with bulbar palsy unassociated with easy fatigability].

A 69-year-old Japanese female was admitted because of progressive nasal voice and dysphagia. Neurological examination revealed paresis of the soft palate with marked dysphagia and rhinolalia. Otherwise there was no weakness or easy fatigability in extraocular muscles and extremities. On laboratory test, anti-acetylcholine receptor antibody (anti-AChR Ab) was positive, while anti-muscle-specific tyrosine kinase antibody (anti-MuSK Ab) was negative. Edrophonium test was positive, resulting in clear improvement in phonation and swallowing. Harvey-Masland test of ocular and extremity muscles did not show any waning. With the diagnosis of bulbar myasthenia gravis, the patient was treated with methylprednisolone and pyridostigmine, resulting in clear improvement of the symptoms. The present case shows that it is important to consider MG even in cases presenting solely with progressive bulbar palsy without easy fatigability. So far, cases of bulbar myasthenia gravis with positive anti-MuSK Ab have often been reported. As shown in the present case, bulbar myasthenia gravis can also be associated with positive anti-ACh-R Ab but negative anti-MuSK Ab.

[Elderly case of moyamoya disease presenting with hemichorea].

A 61-year-old Japanese female was admitted with sudden onset of choreic movements of the right extremities. MRI demonstrated no abnormality suggestive of acute infarcts. Cerebral angiography revealed high-grade stenosis of bilateral middle cerebral arteries at the origin and abnormal vascular network compatible with moyamoya disease. Administration of low-dose haloperidol rapidly resolved the choreic movements. SPECT obtained one month after the clinical onset demonstrated increase of the regional cerebral blood flow (rCBF) in the left basal ganglia. Moyamoya disease presenting chorea as its initial symptom was only infrequently reported in the elderly. In the present case, increased rCBF in the basal ganglia and remarkable effect of a dopamine D2 blocker suggest functional abnormality of the corresponding striatum as an underlying cause of hemichorea.

Transient myoclonic state with asterixis: primary motor cortex hyperexcitability is correlated with myoclonus.

OBJECTIVE: To clarify the clinical features and mechanism of the transience of myoclonus in patients with a transient myoclonic state with asterixis (TMA). METHODS: We investigated the clinical and eletrophysiological profiles of 6 patients with TMA (age: 84±3 years). During an asymptomatic period, somatosensory evoked potentials (SEPs) were recorded in all 6 patients and motor evoked potentials (MEPs) were examined in 1 patient. SEPs were recorded and jerk-locked back averaging (JLA) was performed in 2 patients while symptomatic. SEPs were also recorded from 8 aged control subjects (age: 68±5 years). RESULTS: All TMA patients had mild chronic systemic diseases. During an asymptomatic period, SEP amplitudes were not significantly enlarged in comparison with control subjects, and MEPs were normal. Examination of 2 patients during symptomatic period indicated no enlargement of SEP amplitudes and JLA disclosed a positive spike preceding myoclonic jerks. In one of these patients, the amplitude of the positive spike decreased once myoclonus improved. CONCLUSION: TMA occurred in aged patients with mild chronic systemic diseases. JLA findings and the absence of giant SEPs further support that TMA is a cortical non-reflex myoclonus. In addition, transient hyperexcitability at the primary motor cortex disclosed by JLA correlated well with its transient symptoms.

Left anterior temporal cortex actively engages in speech perception: A direct cortical stimulation study.

Recent neuroimaging studies proposed the importance of the anterior auditory pathway for speech comprehension. Its clinical significance is implicated by semantic dementia or pure word deafness. Neurodegenerative or cerebrovascular nature, however, precluded precise localization of the cortex responsible for speech perception. Electrical cortical stimulation could delineate such localization by producing transient, functional impairment. We investigated engagement of the left anterior temporal cortex in speech perception by means of direct electrical cortical stimulation. Subjects were two partial epilepsy patients, who underwent direct cortical stimulation as a part of invasive presurgical evaluations. Stimulus sites were coregistered to presurgical 3D-MRI, and then to MNI standard space for anatomical localization. Separate from the posterior temporal language area, electrical cortical stimulation revealed a well-restricted language area in the anterior part of the superior temporal sulcus and gyrus (aSTS/STG) in both patients. Auditory sentence comprehension was impaired upon electrical stimulation of aSTS/STG. In one patient, additional investigation revealed that the functional impairment was restricted to auditory sentence comprehension with preserved visual sentence comprehension and perception of music and environmental sounds. Both patients reported that they could hear the voice but not understand the sentence well (e.g., heard as a series of meaningless utterance). The standard coordinates of this restricted area at left aSTS/STG well corresponded with the coordinates of speech perception reported in neuroimaging activation studies in healthy subjects. The present combined anatomo-functional case study, for the first time, demonstrated that aSTS/STG in the language dominant hemisphere actively engages in speech perception.