Intravenous administration of tissue plasminogen activator (t-PA) can improve clinical outcome in patients with acute ischemic stroke. In our country, use of t-PA for acute brain infarction within 3 hours of onset was approved by Japanese government from October, 2005. About 5,700 patients were treated with t-PA for these two years. Analysis of 2,484 patients (mean 70 years old, median NIHSS Score 15) showed that mRS 0-1 was 32%, the death was 20% and symptomatic brain hemorrhage was 5.2%. We had 63 patients (median 74 years old, median NIHSS score 14) treated with t-PA thrombolysis by November, 2007. Immediately after t-PA therapy 8 patients (12.7%) had dramatic recovery. On day 7 after t-PA therapy, excellent recovery was 49.2%, good recovery was 15.9%, and worsening was 12.7%. Within one hour after t-PA therapy, rate of recanalization for occluded arteries was 43.5%, which was strongly associated with excellent and good neurological recovery on day 7. Atrial fibrillation was an independent factor associated with no early recanalization. When we evaluated baseline DWI findings before t-PA infusion using DWI-ASPECTS and NIHSS score at day 7 after rt-PA therapy, bad outcome was seen more frequently in patients with an DWI ASPECTS ≤5 (6 of 8 patients) than in patients with an DWI ASPECTS >5 (2 of 41 patients; P<0.0001). Patients with an ASPECTS-DWI >5 should be considered eligible for t-PA therapy.

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A 26-year-old woman noticed gradually progressive, right lower leg weakness over a 1.5-month period. Neurological examination revealed right hemiparesis with slightly increased deep tendon reflexes, Babinski's sign on the right side, loss of position sense in the right leg, and slight loss of superficial sensation in the right toes. MR FLAIR images showed a high intensity area measuring 5×2×3cm in the left frontal lobe, extending to the outer surface of the body of the corpus callosum and the adjacent right cingulate gyrus. Gadolinium enhancement was seen along the cortex and the outer surface of the body of the corpus callosum. CSF findings showed no pleocytosis, a protein content of 32mg/dl, a sugar level of 85mg/dl, and an IgG index of 0.46.
The biopsy specimen obtained from the superior frontal gyrus showed perivascular cuffing of T-lymphocytes and some B-lymphocytes, as well as multiple small foci of demyelination. Starting on the second day of admission, the patient was treated with methylprednisolone pulse therapy (1,000mg/day for 3 days); she was then switched to oral prednisolone (20mg/day). Thereafter, the patient had two clinical relapses: one was due to a lesion in the dorsal part of the medulla oblongata associated with a disturbance of deep sensation in both hands, and the other was due to a lesion involving the right internal capsule, the globus pallidus, and the caudate nucleus associated with left facial nerve palsy. Visual evoked potentials suggested a demyelinating lesion in the right optic nerve. We suspected a diagnosis of multiple sclerosis based on the presence of more than two clinical episodes of neurological deficits with identifiable lesions on MRI.
Multiple sclerosis should be considered in the differential diagnosis of lesions located in the outer part of the corpus callosum and transcallosal bilateral hemispheres on MRI, even though inner callosal lesions are common in multiple sclerosis.

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We report a 62-year-old woman with intravascular lymphomatosis (IVL) which presented as subacute encephalopathy. She was admitted to our hospital because of loss of consciousness in the middle of February, 2006. Laboratory tests indicated elevated serum C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), and cerebrospinal fluid protein. Magnetic resonance imaging (MRI) of the brain revealed multiple infarct-like lesions mainly in the white matter. After admission, her consciousness was soon improved, but the inflammatory response did not disappear with any antibiotics or virucides. Her consciousness was not exacerbated, and she was discharged in the middle of March, although the reason for loss of consciousness remained unknown.
After discharge she developed an abnormal behavior and mental deterioration, and therefore she was readmitted late in March. On second admission, her consciousness was drowsy. Neurological examinations revealed conjugate deviation of her eyes to the left, left hemiparesis, and generalized hyporeflexia. Laboratory tests showed more elevated CRP than that of the last time, and raised soluble IL-2 receptor (sIL-2R). The repeated MRI of the brain disclosed that initial lesions of the white matter progressively enlarged and increased in number. To make an appropriate diagnosis of the lesions on the brain MRI, the open brain biopsy was performed. Microscopic examination showed that many small vessels were occluded by lymphoma cells (B-lymphocytes) with hemorrhage, and IVL was diagnosed. She was treated with regimens of combined chemotherapy with R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone). After chemotherapy her consciousness and left hemiparesis were gradually improved and the levels of CRP were normalized. The infarcts-like lesions detected on the brain MRI became reduced and decreased.
IVL is a rare disease, and the prognosis is generally poor, with a rapidly fatal outcome, leading to a postmortem diagnosis. In the present report, we successfully treated the patient by rituximab in addition to standard CHOP therapy. Rituximab may play an important role in the treatment of IVL.

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We present herein two patients with bilateral vocal cord paralysis that occurred during the subacute phase of brain infarction. Patients were a 73-year-old man and an 82-year-old woman who suffered from infarction of the basilar artery and the right middle cerebral artery, respectively. The former was diagnosed as atherothrombotic infarction, but the patient experienced repeated aggravation. The latter was diagnosed as cardioembolic infarction. After both patients took clopidogrel and warfarin for the secondary prevention of stroke, upper airway obstruction developed at Day 29 and Day 19, respectively. Vocal cords in Case 1 did not show any movement on laryngoscopy, and were fixed together nearly closed. In Case 2, vocal cords were again almost fixed together. Bilateral vocal cord paralysis is a common complication of cervical operations, but is rare after ischemic stroke. As patients who have suffered from bilateral vocal cord paralysis are often facing death, we must be careful of wheezing with ischemic stroke.

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A 65-year-old man presented with bilateral postural hand tremor (3Hz) followed by memory impairment, which improved spontaneously after admission to our hospital. Two weeks after admission, he had another bout of memory impairment and complained of insomnia. Brain magnetic resonance imaging (MRI) showed a signal intensity change in the medial part of the right temporal lobe, which suggested limbic encephalitis (LE). Serum hyponatremia and absence of cerebrospinal fluid pleocytosis suggested the possibility of anti-voltage-gated potassium channel (VGKC) antibody-associated LE. Serum hyponatremia and hand tremor showed immediate improvement after steroid pulse therapy, and memory impairment and insomnia showed gradual improvement. Brain MRI on day 110 of admission showed partial resolution of medial temporal signal abnormalities with mild bilateral hippocampal atrophy. Serological tests confirmed the clinical diagnosis of anti-VGKC antibody-associated LE. This report may expand the phenotypic profile of anti-VGKC antibody-associated LE, which is characterized by remission and relapse of disease course, insomnia, and hand tremor.

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We reported two patients with unilateral lateral medullary infarction involving severe, long-term respiratory failure. The first patient is an 86-year-old man presenting with gait disturbance, hoarseness and dysphagia. A right lateral medullary infarction was revealed by brain MRI. On the fifth hospitalized day, acute respiratory failure occurred. His condition failed to recover, and he was still attached to a ventilator 10 months after the onset. The second patient is an 83-year-old woman mainly presenting with dysphagia. A tiny infarction in the right lateral medulla was revealed by brain MRI. On the third day after the onset, acute respiratory failure occurred, which was not changed even at 8 months later. Although the symptoms and the lesion in the medulla were quite different between two patients, dysphagia and respiratory failure occurred in both patients. Therefore, it is postulated that the lesion that causes dyspnea may be approximate to the lesion that causes dysphagia. Many previously reported cases presenting respiratory failure seemed to suffer swallowing difficulty as well. We conclude that elderly patients suffering from unilateral lateral medullary infarction with dysphagia can present respiratory failure a few days after the onset, demonstrating the need to observe them under intensive attention.

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A 49-year-old man noticed a resting tremor in his right hand and was diagnosed with Parkinson's disease (PD) at the age of 43. Soon thereafter, he noticed a right neck mass that only appeared when he swallowed and was accompanied by an uncomfortable sensation. At the age of 49, he was admitted to our hospital to adjust his medication for PD and to examine the neck mass. The subcutaneous mass was round, soft, and approximately 3cm in diameter. The symptoms in his neck showed no progression. Resting tremors and rigidity due to his PD were predominantly observed on his right side, but his postural reflex was intact. Cervical echogram revealed that the mass was the belly of the OM itself. Thus, the patient was diagnosed with omohyoid muscle syndrome (OMS). It can be surmised that some susceptibility of the OM itself and/or structural fragility in the surrounding tissues would be involved in the pathogenesis of OMS. In this case, considering that OMS occurred soon after symptoms of PD appeared, we speculated that muscle tone abnormalities due to PD played a role in the development of OMS.

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An 62-year-old man presented visual impairment and generalized seizure. Brain CT performed on the day of admission showed thrombus in the right transverse sinus, and DWI showed high intensity areas in the bilateral occipital and parietal lobes. According to bilateral occipital lobe lesions, we considered his visual impairment as cortical blindness. He was diagnosed as venous sinus thrombosis and intravenous heparin, edaravone and osmotic diuretics were administered. MR venography performed after starting of intravenous treatment showed flow gap in the left transverse sinus but no abnormalities in the right transverse sinus. On the second day of hospitalization, his cortical blindness showed improvement and thrombus in the right transverse sinus were disappeared. This indicated that his left transverse sinus originally hypoplastic, thrombus and hemostatis in the right transverse sinus (his dominant side) caused his cortical blindness and generalized seizure. There was a recanalization in the right transverse sinus after heparin therapy.

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A 56-year-old woman attended our hospital because of acute severe (thunderclap) headache. Neurological examination was normal and no abnormality was found on head CT or by cerebrospinal fluid examination. A few days later, she experienced a recurrence and suffered a seizure in her left upper and lower extremities. On neurological examination, she had conjugate deviation of the eyes toward the right side and left lower limb paralysis with Chaddock sign. MRI showed multiple hyperintense lesions in the bilateral occipital and parietal lobes, predominantly in the subcortical white matter at the right side on T2-weighted and FLAIR images. We diagnosed posterior reversible encephalopathy syndrome (PRES) because the ADC map showed a vasogenic edema pattern (increased ADC values in the hypodense lesions on diffusion-weighted image). Her blood pressure was normal and there were no underling diseases. As MRA showed vasoconstriction especially in bilateral posterior cerebral arteries, we initiated a therapy with a Ca-channel blocker. On follow-up MRI, the hyperintense lesions on T2-weighted and FLAIR images had almost disappeared, and vasoconstriction was also improved on MRA. This case suggested that cerebral vasoconstriction could underlie both thunderclap headache and PRES.

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We report a patient of Charcot-Marie-Tooth disease (CMT) accompanied by transient splenium abnormality in brain MRI. A 34-year-old man suffered from chronic progressive unsteadiness and sensory disturbance of all limbs. Neurological examination showed muscle weakness and atrophy in the distal extremities with pes cavus, mild sensory disturbance of four extremities and generalized decreased reflexes. The nerve conduction study described the presence of sensory-motor polyneuropathy. We could not investigate his GJB1 gene. However, we suspected that he was X-linked CMT (CMTX), because his electrophysiological findings showed intermediate slowing of MCV, and auditory brain-stem response (ABR) demonstrated central conduction slowing. Brain MRI revealed the abnormal high signal intensity in the splenium of the corpus callosum on T2-weighted image. This lesion diminished two months later without any treatment.
Recently, there had been reported transient splenium abnormality in CMTX cases, and there were clinical similarities between the cases of these reports and our case. We considered that the pathophysiology of this case was the disruption of gap junction communications expressed between oligodendrocyte and astrocytes induced by connexin 32 (Cx32) mutations. Furthermore, the transient functional disturbance of astrocytes would be another pathophysiologic mechanism of splenium abnormality.

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