Neuronal intranuclear inclusion disease (NIID) is a neurodegenerative disease presenting with various manifestations including dementia, weakness, transient impaired consciousness, encephalitis-like episodes and also epileptic seizures. However, the nature of epileptic seizures, focal or generalized onset, remains unclear. A man at age 76 was admitted to a local hospital due to febrile impaired consciousness lasting several days. During the hospital stay, a generalized convulsion occurred, and afterward he remained obtunded. He was transferred to our hospital for further treatment. One additional seizure occurred while on an ambulance to our hospital and two additional seizures shortly after the arrival, which indicated convulsive status epilepticus (SE). The ictal EEG showed low amplitude fast activity arising from the left centro-parietal area with an evolutionary pattern. The clinical concomitant was the contralateral versive seizure evolving to a bilateral convulsion. Inter-ictal epileptiform abnormalities seen on the tracings taken on later days consisted of brief potentially ictal rhythmic discharges (B(I)RDs) and frequent sharp waves recorded from both frontal areas. These findings along with the ictal discharges would indicate a multifocal epileptic disorder in this patient. Diffusion weighted images (DWIs) of this patient showed hyperintensity signals in the cortico-medullary junctions in the bilateral frontal and the left parietal regions. Skin biopsy revealed characteristic intranuclear inclusions, and hence the diagnosis of NIID was made. The regions of epileptic foci on EEG corresponded well to the hyperintensity areas in DWIs, which suggests the cerebral cortices near the DWI hyperintensity could be more hyperactive than other regions. This case report suggests that some of the prolonged impaired consciousness and the encephalitis-like episodes in NIID could be related to epileptic seizures and even non-convulsive SE or a post-ictal twilight state after an unwitnessed convulsion. This issue should be further studied for the appropriate treatment of episodic encephalopathy and epileptic seizures in NIID.

A 63-year-old man presented with right-sided hemianopia and unsteady gait. Brain MRI revealed multiple hyperintense infarct-like lesions on diffusion-weighted images (DWI). Hyperintensity persisted in some of these lesions even after 6 weeks, although his symptoms were ameliorated then. The patient developed episodic dizziness and a transient event of apraxia at 18 weeks after the first episode. Brain MRI revealed additional hyperintense lesions on DWI, which persisted even after 7 weeks. Eventually, the patient manifested cauda equina syndrome 39 weeks after the first episode. Brain MRI showed the presence of new lesions in addition to the persistent hyperintense lesions on DWI over 21 weeks in the right frontal lobe. Based on laboratory findings and the pathological assessment of bone marrow and random skin biopsies, the patient was diagnosed with intravascular lymphoma (IVL). Persistent hyperintense lesions on DWI of brain MRI may precede the clinical exacerbation of IVL.

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Introduction: Detection of systemic embolization is important for treating patients with infective endocarditis (IE). Subclinical small emboli, many of which might occur behind the overt embolization, will be detected as a microembolic signal (MES) with transcranial Doppler (TCD). Case report: A 54-year-old male with fever and impaired consciousness was admitted with suspected encephalopathy. His medical history included bioprosthetic aortic valve replacement performed 3 years before admission. Although IE was considered as differential diagnosis, transthoracic echocardiography and contrast-enhanced CT scan did not reveal the presence of lesions. According to the modified Duke criteria, his case was classified as "rejected IE." However, TCD revealed four MESs in the left middle cerebral artery, and IE was reconsidered. Transesophageal echocardiography revealed a 14-mm mobile vegetation on the prosthetic valve. Although early surgery was planned, CT revealed small subarachnoid hemorrhages in the left cerebral hemisphere. The risk of systemic embolization was considered high; therefore, valve replacement was performed at day 6 after admission. After 1 month, the patient was discharged without sequelae. Conclusions: MESs may be useful as a marker for subclinical embolization in patients with IE. Further studies should assess the potential of TCD for the diagnosis and risk stratification of IE.