Two Cases of Alice in Wonderland Syndrome with a Right Occipital Lobe Lesion Caused by Isolated Cortical Venous Thrombosis.

Alice in Wonderland syndrome (AIWS) is extremely rare, occurring more often in young individuals than in older adults. Symptoms of this syndrome typically include an altered body image, size perception, and time perception. However, the pathophysiology and lesions responsible for this syndrome remain unclear. In most cases, specific lesions cannot be identified using computed tomography or magnetic resonance imaging. Two patients with isolated cortical venous thrombosis in the right occipital area experienced transient visual symptoms of AIWS. Furthermore, a literature search indicated that AIWS with visual distortions is associated with right occipital lobe lesions, supporting the findings of our study.

A 5-Minute Delay From Bolus Injection to Infusion Halves Plasma Concentration of Tissue Plasminogen Activator.

BACKGROUND: Reducing the time between onset of cerebral infarction and treatment with tissue plasminogen activator improves the prognosis of patients with cerebral infarction. Diverse dosing protocols have been developed with the aim of reducing the time to bolus injection; however, only a few studies have investigated the methods and effects of the interrupted time between bolus and post-bolus infusion. OBJECTIVE: We evaluated the impact of the interrupted time on pharmacokinetic parameters. METHODS: We calculated the changes in alteplase concentration after a bolus injection with high precision, in relation to different interval times. Simulations were performed using the linpk package of the statistical analysis software R. Post-bolus infusion was initiated at 0-, 5-, 15-, and 30-min intervals after bolus dosing. The calculation interval was set as 6 s. RESULTS: Alteplase concentration rose to 1.23 mg/mL after bolus dosing. However, it dropped to 0.53 mg/mL (43.4%) during a 5-min interval, 0.27 mg/mL (22.23%) during a 15-min interval, and 0.10 mg/mL (8.38%) during a 30-min interval. CONCLUSIONS: Because of the short half-life of alteplase, even a short delay in initiating post-bolus infusion can cause a significant reduction in serum alteplase concentration.

Lung Lobectomy Is an Embolic Risk Factor for Cerebral Infarction, Even in the Long-term Postoperative Phase.

The vein stump created after lung lobectomy has been reported to cause thrombus and subsequently cerebral infarction. However, its assessment after a long-term postoperative course remains unreported. The pulmonary vein stump is a structure near the left atrial appendage; therefore, such patients may be at a constant risk of thrombus formation. We herein report two cases of cerebral infarction associated with lung lobectomy. Transesophageal echocardiography revealed mobile thrombi in the pulmonary vein stump. Both patients had cancer recurrence, and hypercoagulability may have contributed to thrombus formation. This vein stump should be investigated as an embolic source, even after a long postoperative duration.

A case of encephalitis following COVID-19 vaccine.

We describe the first case of encephalitis following coronavirus disease 2019 (COVID-19) vaccination. Our patient was a 46-year-old Japanese woman who presented with acute onset diplopia. Subsequent magnetic resonance imaging revealed brain stem encephalitis that was rapidly responsive to high dosage steroid therapy and completely improved. Although the occurrence of encephalitis after vaccination could have just been a casual temporal association, her symptoms were temporally correlated with two vaccinations. Our case suggests caution and indicates treatment and prognosis, despite no evidence of a causal relationship. Nonetheless, this report emphasizes the enormous benefits of vaccination, which should not be undermined.

EEG-EMG polygraphic study of dystonia and myoclonus in a case of Creutzfeldt-Jakob disease.

We report on a patient with sporadic Creutzfeldt-Jakob disease (CJD) who showed dystonia, periodic myoclonus, and periodic sharp wave complexes (PSWCs) on EEG. The EEG-EMG polygraphic study revealed that dystonia appeared without relation to periodic myoclonus and PSWCs and that dystonia EMGs were strongly suppressed after periodic myoclonus EMGs. These findings suggest that dystonia has a pathogenesis different from that of periodic myoclonus and PSWCs, but dystonia and periodic myoclonus may be generated through the sensorimotor cortex in CJD.

Experimental and theoretical study on the excited-state dynamics of ortho-, meta-, and para-methoxy methylcinnamate.

The S1 state dynamics of methoxy methylcinnamate (MMC) has been investigated under supersonic jet-cooled conditions. The vibrationally resolved S1-S0 absorption spectrum was recorded by laser induced fluorescence and mass-resolved resonant two-photon ionization spectroscopy and separated into conformers by UV-UV hole-burning (UV-UV HB) spectroscopy. The S1 lifetime measurements revealed different dynamics of para-methoxy methylcinnamate from ortho-methoxy methylcinnamate and meta-methoxy methylcinnamate (hereafter, abbreviated as p-, o-, and m-MMCs, respectively). The lifetimes of o-MMC and m-MMC are on the nanosecond time scale and exhibit little tendency of excess energy dependence. On the other hand, p-MMC decays much faster and its lifetime is conformer and excess energy dependent. In addition, the p-MMC-H2O complex was studied to explore the effect of hydration on the S1 state dynamics of p-MMC, and it was found that the hydration significantly accelerates the nonradiative decay. Quantum chemical calculation was employed to search the major decay route from S1(ππ(∗)) for three MMCs and p-MMC-H2O in terms of (i) trans → cis isomerization and (ii) internal conversion to the (1)nπ(∗) state. In o-MMC and m-MMC, the large energy barrier is created for the nonradiative decay along (i) the double-bond twisting coordinate (∼1000 cm(-1)) in S1 as well as (ii) the linear interpolating internal coordinate (∼1000 cm(-1)) from S1 to (1)nπ(∗) states. The calculation on p-MMC decay dynamics suggests that both (i) and (ii) are available due to small energy barrier, i.e., 160 cm(-1) by the double-bond twisting and 390 cm(-1) by the potential energy crossing. The hydration of p-MMC raises the energy barrier of the IC route to the S1/(1)nπ(∗) conical intersection, convincing that the direct isomerization is more likely to occur.

A hemorrhagic pineal cyst with a bacterial meningitis-like manifestation and benign outcome.

Pineal cysts are a common incidental finding in imaging studies, and the majority of such cysts are asymptomatic. However, hemorrhaging pineal cysts, which are considered to be rare, are often associated with severe symptoms. We herein describe the case of a 58-year-old patient with the novel manifestation of a bleeding pineal cyst, who had a benign outcome without any surgical treatment. Although the clinical manifestations resembled those of bacterial meningitis, magnetic resonance images suggested chemical meningitis caused by an intracystic hemorrhage and rupture of the pineal cyst.

Rituximab Improves Subclinical Temporal Dispersion of Distal Compound Muscle Action Potential in Anti-MAG/SGPG Neuropathy Associated with Waldenström Macroglobulinemia: A Case Report.

Patients with anti-myelin-associated glycoprotein (MAG)/sulfated glucuronyl paragloboside (SGPG) neuropathy associated with Waldenström macroglobulinemia show demyelinating neuropathy, but the temporal dispersion of distal compound muscle action potential (CMAP) in motor nerve conduction studies (NCS), which represents heterogeneous demyelination at the motor nerve terminal, is rare. We report on a 70-year-old man with anti-MAG/SGPG neuropathy associated with Waldenström macroglobulinemia; he had a 2-year history of mild dysesthesia of the foot sole without any motor symptoms. He showed marked temporal dispersion of distal CMAP in the tibial nerve with other demyelinating findings in the NCS. The temporal dispersion of distal CMAP in the tibial nerve improved significantly, and motor function was again normal 1 year after rituximab monotherapy. The temporal dispersion of distal CMAP in anti-MAG/SGPG neuropathy is rare, but it could occur from an early stage when the patients show mild or no motor symptoms. Rituximab therapy before secondary axonal degeneration has great potential to reverse the effects of the demyelination including the temporal dispersion of distal CMAP, and to prevent the deterioration of neuropathy in anti-MAG/SGPG neuropathy.

First attack of Kleine-Levin syndrome triggered by influenza B mimicking influenza-associated encephalopathy.

Six days after the onset of influenza B symptoms, a 14-year-old Japanese boy presented with encephalopathy-like symptoms, somnolence, irritability, and childishness, which we first considered was an atypical type of influenza-associated encephalopathy because the infection symptoms disappeared by day 4. His encephalopathy-like symptoms gradually improved, although he had repetitive hypersomnia attacks. Owing to the patient's clinical presentation and normal interleukin-6 levels in the cerebrospinal fluid during the first period of hypersomnia, we diagnosed him with Kleine-Levin syndrome (KLS) triggered by influenza B. The preceding influenza infection was not only a diagnostic clue of KLS but also a diagnostic confounding factor.

Reversible extensive leukoencephalopathy in Sweet disease: a case report.

Sweet disease, also known as acute febrile neutrophilic dermatosis, is an idiopathic multisystem inflammatory disorder characterised by erythematous skin lesions and fever. "Neuro-Sweet disease" is a rare central nervous system involvement that coexists with the characteristic cutaneous lesions of Sweet disease. Here, we report a case of Sweet disease complicated with acute encephalopathy. This case showed extensive lesions in the cerebral white matter on magnetic resonance imaging (MRI) and high protein concentration in the cerebrospinal fluid (CSF) without pleocytosis. After steroid therapy, the patient's clinical syndrome recovered completely with no significant neurological deficits or abnormal findings on brain MRI, and normalization of the high CSF protein concentration. Both clinical and MRI findings suggested that the marked leukoencephalopathy in this case was mainly due to reversible oedema rather than destructive structural alterations in the cerebral parenchyma.

Rituximab therapy in chronic inflammatory demyelinating polyradiculoneuropathy with anti-SGPG IgM antibody.

We report a patient with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) who showed high titers of anti-sulfated glucuronyl paragloboside (SGPG) IgM antibody without M-protein in serum. The patient was resistant to corticosteroids and immunosuppressants, but after administration of rituximab, clinical symptoms improved and the patient remained in a stable state for approximately 10 months. Rituximab may be a potent therapeutic option for refractory cases of CIDP irrespective of detectable M-protein in either serum or urine.

High-dose melphalan followed by autologous stem cell support in primary systemic AL amyloidosis with multiple organ involvement.

A patient with primary systemic AL amyloidosis achieved partial hematological response after 2 courses of VAD (vincristine, doxorubicin and dexamethasone) and subsequent high-dose melphalan followed by autologous peripheral blood stem cell transplantation despite involvement of multiple organs, including the heart. In this patient natriuretic peptides and free light chains in serum were useful as markers of cardiac involvement and therapeutic effects, respectively. When amyloidosis-related dysfunction is seen in multiple organs, this intensive chemotherapy might be a possible therapeutic option, although several modifications in the regimen and careful management are necessary.

Chronic inflammatory demyelinating polyradiculoneuropathy with autonomic involvement.

We report a case of chronic acquired neuropathy predominantly affecting sensory and autonomic nerves. Investigations showed a demyelinating polyradiculoneuropathy with axonal degeneration and depletion of postganglionic noradrenergic fibers in the rectal mucosa. Intravenous immunoglobulin and corticosteroid administration were effective in alleviating symptoms and improving electrophysiological abnormalities. This neuropathy may be a novel variant of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), in which autoimmunoreactivity is directed not only against myelin but also against axon- or ganglion-composing protein. Autonomic nerve involvement does not exclude a diagnosis of CIDP.