Opalski Syndrome Treated with Intravenous Recombinant Tissue Type Plasminogen Activator-Case Report and Review of Literature.

A 65-year-old man with a history of Wallenberg syndrome caused by vertebral artery dissection at 62 years old was admitted to our hospital with nausea, vertigo, right facial dysesthesia, right hemiplegia, crossed sensory disturbance (sensory loss and numbness in the right face and left body below the neck), and right limb ataxia. Magnetic resonance imaging (MRI) performed 80 minutes after onset revealed no acute ischemic stroke lesions, but magnetic resonance angiography (MRA) demonstrated complete occlusion of the right vertebral artery. Based on these neurological and MRA findings, atypical lateral medullary infarction was suggested, and intravenous tissue plasminogen activator (IV-tPA) was started 178 minutes after onset. Right hemiplegia improved immediately after IV-tPA administration. MRI performed on hospital day 2 showed an acute ischemic lesion on the right side of the medulla oblongata, resulting in a diagnosis of Opalski syndrome. Opalski syndrome is a rare subtype of Wallenberg syndrome accompanied by hemiplegia of the side ipsilateral to the lesion, and expansion of the stroke lesion to the corticospinal tract below the pyramidal decussation is considered to cause ipsilateral hemiplegia. Based on this case and previous reports, Opalski syndrome should be considered when limb ataxia and crossed sensory deficit are observed among patients with hyperacute-onset hemiplegia, and IV t-PA therapy should be considered even in the absence of neurological findings such as dysphagia, dysarthria, and Horner's signs and radiological evidence of acute ischemic stroke.

A Case of Subacute Combined Degeneration of Spinal Cord Diagnosed by Vitamin B12 Administration Lowering Methylmalonic Acid.

Subacute combined degeneration of the spinal cord (SCDS) is a neurodegenerative disease characterized by subacute progression in the central and peripheral nervous systems mainly caused by vitamin B12 deficiency. It is known that typical SCDS is frequently accompanied by megaloblastic anemia and increased serum methylmalonic acid (MMA) or homocysteine (Hcy) levels on laboratory findings, and marked abnormalities on spinal cord magnetic resonance imaging (MRI). A 45-year-old woman was admitted to our hospital with a 2-year history of worsening mild weakness, numbness in bilateral lower limbs, and gait disturbance. On admission, as laboratory findings, blood count showed macrocytosis without anemia, and biochemical tests showed mild reduction in total serum vitamin B12 level and no increase of MMA and Hcy levels; there were no abnormal findings on spinal cord MRI. After administration of vitamin B12, her sensorimotor symptoms were improved and laboratory examination showed that macrocytosis was improved, serum vitamin B12 was increased, and serum MMA levels were decreased. This improved clinical course and the laboratory findings following vitamin B12 administration confirmed the diagnosis of SCDS due to vitamin B12 deficiency. SCDS presents with highly variable symptoms and laboratory findings, and observation of MMA levels and neurologic symptoms before and after vitamin B12 administration may be useful for diagnosing SCDS.

[An adult onset sporadic neuronal intranuclear inclusion disease case reminiscent with Fisher syndrome].

A 63-year-old woman presented to our hospital with sudden symptoms of unsteadiness while walking. Based on the neurological findings, i.e., ataxia and absence of tendon reflex in the extremities accompanied by antecedent infection at the time, she was tentatively diagnosed with Fisher syndrome. Following intravenous immunoglobulin (IVIg) therapy for 5 days, her ataxic symptoms improved. Laboratory data were negative for antiganglioside antibody against GQ1b in the IgG subclass. Six months after her first admission, cognitive impairment gradually developed. She was re-admitted owing to new onset of unsteadiness while walking 1.5 years after her first admission. Diffusion-weighted brain MRI (DWI) revealed linear high-intensity signals in the region of the corticomedullary junction. Cutaneous skin biopsy revealed intranuclear inclusion bodies in sweat gland cells. Considering her family history along with the examination results, we diagnosed with adult-onset sporadic neuronal intranuclear inclusion disease (NIID). Retrospective investigation of the previous DWI obtained at the first admission had also shown slight linear high-intensity areas, suggesting that a series of events, including repeated sudden-onset transient ataxia, resulted due to NIID.

Association between paroxysmal atrial fibrillation and the left atrial appendage ejection fraction during sinus rhythm in the acute stage of stroke: a transesophageal echocardiographic study.

BACKGROUND: This study aimed to investigate whether left atrial appendage (LAA) dysfunction evaluated by transesophageal echocardiography (TEE) during sinus rhythm is predictable of paroxysmal atrial fibrillation (PAF) as an embolic source in the acute stage of stroke. METHODS AND RESULTS: We measured and analyzed LAA flow velocity (LAA-FV) and LAA ejection fraction (LAA-EF) in 300 acute ischemic stroke patients by TEE. We divided the acute ischemic stroke patients into 3 groups. The atrial fibrillation (AF) group (n=58) comprised patients whose TEE was performed during AF rhythm. The PAF group (n=42) comprised patients with a history of AF but with normal sinus rhythm when TEE was performed. The normal sinus (sinus) group (n=200) did not have any history of AF. We found that mean LAA-FV and LAA-EF values in the PAF group were significantly lower than those in the sinus group (P<.001). The diagnostic accuracy of LAA-FV for the diagnosis of PAF calculated as the area under receiver operating characteristic curves was low (.582, 95% confidence interval [CI]=.498-.665) but that of LAA-EF was modest (.721, 95% CI=.653-.789), with an optimal cutoff point of 49.1%. CONCLUSIONS: LAA dysfunction as determined by TEE (LAA-EF<49.1%) in the acute stage of stroke is predictive of PAF with moderate accuracy. Long-term electrocardiographic monitoring is recommended for cryptogenic stroke patients with LAA dysfunction.

Association between inflammatory biomarkers and progression of intracranial large artery stenosis after ischemic stroke.

BACKGROUND: Proinflammatory state has been implicated as a pathogenetic mechanism in the progression of intracranial large artery atherosclerosis (ILA). High levels of inflammatory biomarkers in healthy populations and in patients with acute stroke or acute coronary syndrome are known to be associated with subsequent stroke events. This study investigated the relationship between circulating biomarkers measured early after stroke onset and future ILA progression. METHODS: In 48 patients with acute ischemic stroke, high-sensitivity C-reactive protein (hs-CRP), interleukin-6 (IL-6), IL-18, tumor necrosis factor-α, matrix metalloproteinase (MMP)-2 and MMP-9 were measured within 48 hours after onset. Baseline severity and ILA progression were assessed by serial magnetic resonance angiography (MRA). The median follow-up period for MRA was 3.1 years. Hazard ratio (HR) was calculated using the Cox proportional hazard model adjusted for traditional risk factors, and accuracy of predicted ILA progression was analyzed by receiver operating characteristic (ROC) curve analysis. RESULTS: ILA progression was observed in 6 of 48 patients (12.5%). After adjusting for age, sex, and presence of hypertension, baseline ILA severity score (HR 2.814; 95% confidence interval [CI] 1.172-6.754) and IL-6 (HR 1.215; 95% CI 1.002-1.473) were significantly associated with ILA progression. Area under the ROC curve (AUC) for prediction of ILA progression by traditional risks, baseline ILA severity score and IL-6, was 0.647. When IL-6 was removed from this model, AUC remained at 0.631. CONCLUSIONS: In addition to traditional risk factors and baseline radiologic findings, circulating levels of IL-6 measured soon after stroke onset are associated with future ILA progression.

Effects of edaravone, a free radical scavenger, on serum levels of inflammatory biomarkers in acute brain infarction.

The potent free radical scavenger edavarone is widely used in Japan to treat acute ischemic stroke within 24 hours after onset. Recent experimental studies have shown that edavarone alleviates blood-brain barrier disruption in conjunction with suppression of the inflammatory reaction in acute brain ischemia. We investigated the effects of edaravone on circulating inflammatory biomarkers in patients with ischemic stroke. Patients with acute ischemic stroke admitted 12-36 hours after onset of symptoms were prospectively enrolled. Intravenous edaravone at 60 mg/day for 14 days was administered to patients admitted 12-24 hours after symptom onset (edaravone group; n = 29). Patients admitted 24-36 hours after onset served as controls (control group; n = 34). Venous blood samples were obtained on admission and at 48 hours, 7 days, and 14 days after symptom onset. Serum concentrations of high-sensitivity C-reactive protein, interleukin (IL)-6, IL-10, IL-18, tumor necrosis factor α, matrix metalloproteinase (MMP)-2, and MMP-9 were measured. General linear models were used to compare changes in concentrations of these biomarkers over time between the groups. In the control group, the mean MMP-9 concentration increased gradually from 3.857 ± 1.880 ng/mL to 4.538 ± 1.966 ng/mL over the 14-day period (P = .027, one-way repeated-measures analysis of variance [ANOVA]), but the edavarone group demonstrated no such increase (P = .564). A significant group-time interaction was demonstrated only for MMP-9 (P = .029, two-way repeated-measures ANOVA), and no significant differences in other biomarkers were seen between groups. Our data indicate that edaravone suppresses serum MMP-9 level in patients with acute ischemic stroke. Further studies with a larger sample size are needed to explore the relationship between circulating MMP-9 level and the protective effect of edaravone.

[Effects of early statin treatment on inflammatory biomarkers and clinical deterioration in patients with acute ischemic stroke].

We prospectively studied the effects of early statin treatment on stroke-induced changes in the levels of inflammatory biomarkers. Patients admitted within 48 hours after the onset of ischemic stroke were enrolled. They were divided into 2 groups according to their lipid profiles and history of statin treatment. In patients who had received statin treatment prior to admission and those who had abnormal lipid profiles on admission, daily treatment with 10 mg atorvastatin was initiated within 48 hours after the onset of stroke (Statin group; n = 45). In patients who had normal lipid profiles on admission, statin was not administered for at least 2 weeks after admission (Non-Statin group; n = 101). The serum concentrations of interleukin (IL)-6, IL-10, IL-18, matrix metalloproteinase (MMP)-2, MMP-9, and high sensitive C-reactive protein were measured on days 1, 3, 7, and 14. In percentage changes in serially measured circulating IL-6 levels, a significant interaction between group and repeated measures (group X time factor) was demonstrated (p = 0.047). Frequency of neurological deterioration episodes (NIHSS score > or = 2) during 14 days after admission was lower in the Statin group than in the Non-Statin group, however the difference did not reach statistically significant level (7.9% vs 20.2%, p = 0.118). The initiation of usual dose of atorvastatin early after the onset of ischemic stroke significantly decreased the elevation of IL-6 and may protect against the early neurological deterioration. Circulating levels of IL-6 may be one of the candidates for monitoring the acute effects of statin. Further studies wherein IL-6 levels are monitored in larger samples would be feasible for investigating the effect of early treatment with usual dose of atorvastatin on the functional outcome.

[Case of Parkinson's disease presenting with unique dyspneic attacks caused by oromandibular dystonia and sleep apnea syndrome].

A 79-year-old woman with a 4-year history of Parkinson's disease was admitted due to unique dyspneic attacks with cyanosis while eating. Dyspneic attacks with cyanosis occurred mainly during actions such as taking meals or rehabilitation. Due to increased tonus of the orbicularis oris muscle, she was unable to open her mouth and breathe out, and finally experienced hypoxemia as revealed by pulse oxymetry. Dystonic hypertonus was relieved by touching the mandible with the fingers, and she was able to open her mouth again. These symptom was compatible with the sensory trick. Based on these findings, we considered that dyspneic attacks were produced by focal oromandibular dystonia. Polysomnography also showed central sleep apnea. We report herein a rare case of Parkinson's disease presenting with respiratory insufficiency caused by focal dystonia and central sleep apnea.