Sun-Ah Choi

Distribution of the corticobulbar tract in the internal capsule

It is generally thought that the corticobulbar tract descends through the genu of the internal capsule (IC). There have been several reports that genu lesions cause bulbar symptoms such as facial palsies, dysarthria, and dysphagia. However, the precise location of the corticobulbar tract in the IC remains controversial. The purpose of our study is to assess whether the corticobulbar tract passes through the IC genu. We reviewed 26 patients with selective IC infarction and located the sites related to bulbar symptoms. In addition, using diffusion tensor imaging, we reconstructed tracts passing through the IC in ten subjects without cerebral infarction. Patients with genu infarction, which extended to more than half of the posterior limb of the IC, showed bulbar symptoms. However, patients with genu infarction, which was limited to the genu, did not have bulbar symptoms. In contrast, patients with lesions limited to the posterior limb may show bulbar symptoms. According to statistical maps of the region of interest, the lesions related to bulbar symptoms were localized to areas that were beyond the midpoint of the posterior limb of the IC. In diffusion tensor imaging of subjects without cerebral infarctions, the corticobulbar and corticospinal tracts did not pass through the IC genu. Our data provide evidence that the corticobulbar tract does not pass through the IC genu. The proposed location of the corticobulbar tract in the level of the IC lies beyond the midpoint of the posterior limb.

Preconditioning with Chronic Cerebral Hypoperfusion Reduces a Focal Cerebral Ischemic Injury and Increases Apurinic/Apyrimidinic Endonuclease/Redox Factor-1 and Matrix Metalloproteinase-2 Expression

Atherosclerosis may cause severe stenosis of the arteries supplying the brain, which induces chronic cerebral hypoperfusion. Although an infarction often occurs in the chronically hypoperfused brain area, it has been uncertain whether the stroke severity is attenuated or increased when further decrease of blood flow occurs. To test the hypothesis that chronic cerebral hypoperfusion is protective against the subsequent severe ischemia, we examined the effect of chronic cerebral hypoperfusion on brains subjected to acute focal ischemia. Spontaneous hypertensive rats were subjected to middle cerebral artery occlusion/reperfusion four weeks after bilateral common carotid artery ligation (BCAL) or sham operation. The rats with BCAL had smaller infarctions, determined by 2,3,5-triphenyltetrazolium hydrochloride staining, and less severe neurologic deficits than those with sham operation. The number of DNA-damaged cells, examined by the in situ nick translation study, was significantly reduced in animals with BCAL. Immunoreactivity for apurinic/apyrimidinic endonuclease/redox factor-1, which plays a role in cellular defense mechanism, was markedly increased in those with BCAL. Indirect evidence of extracellular matrix remodeling, which might be associated with adaptive arteriogenesis or angiogenesis, was obtained in the form of increased matrix metalloproteinase-2 activity in them. These findings provide experimental evidence that chronic cerebral hypoperfusion would be protective against subsequent severe ischemic insults.

A case with improvement of blepharospasm by zolpidem.

Zolpidem is usually used for the treatment of insomnia as a hypnotic drug. It was also suggested to be effective in the treatment of dystonia in some studies. A 74-year-old woman had been suffering from frequent and intense bilateral spasms of the eyelids for 20 years. She has been treated with botulinum toxin injection and taken some medications. But, she experienced a little effect and was not satisfied with those treatments. Her symptom was improved after taking Zolpidem which had been prescribed for insomnia by her primary physician. She did not show any improvement after placebo injection and neostigmine test. This is the first report which shows improvement of isolated blepharospasm by Zolpidem in Korea. Zolpidem can be one of useful alternative pharmacological treatments for blepharospasm. Further randomized, blinded, placebo-controlled studies are needed to validate this finding.

Subcortical vascular dementia (SVaD) without hypertension (HTN) may be a unique subtype of vascular dementia (VaD)

Although HTN is the most important factor in the pathogenesis of SVaD, about 20% of patients with SVaD do not have HTN. We hypothesize that SVaD without HTN may have strong risk factors other than HTN, and the study on this group can elucidate the risk factors for SVaD. We included 332 patients with SVaD from the database of the Clinical Research Center for Dementia of South Korea (CREDOS) study. Among them, 26.2% of patients (87 out of 332) had no history of HTN. We analyzed the differences in risk factors, clinical features, and survival time of SVaD according to HTN. Contrary to our expectations, SVaD without HTN had less known vascular risk factors such as diabetes mellitus (DM), dyslipidemia, and obesity. In addition, SVaD without HTN had different clinical features including less depression, focal neurological signs or symptoms and more features of disinhibition. However, although SVaD without HTN had less known vascular risk factors that can shorten survival times, the survival times did not differ according to the presence of HTN. SVaD without HTN may be a unique subtype of SVaD and can be a target group for studies of unknown risk factors for SVaD.

A Case of Isolated Middle Cerebral Artery Stenosis with Hemichorea and Moyamoya Pattern Collateralization

Isolated middle cerebral artery (MCA) stenosis in young patients with no other medical condition may be a unique pathologic entity with a benign long-term course. Generally, moyamoya disease shows a progression of stenosis from internal cerebral artery (ICA) to other intracranial vessel. A 26-year-old woman was admitted for choreic movements of the right arm and leg. Brain magnetic resonance imaging showed no stroke. Conventional angiography revealed 48% stenosis of the left M1 without ICA stenosis. Single photon emission computed tomography revealed perfusion asymmetry after acetazolamide injection, suggesting decreased uptake in the left basal ganglia and the cerebral cortex. Her hemichorea was mildly decreased with risperidone. One year later, follow-up angiography showed complete occlusion of the left M1 with neovascularization suggestive of moyamoya disease. The patient underwent bypass surgery and her hemichorea disappeared. This may be an atypical presentation of moyamoya disease. The bypass surgery was an effective measure for restoring the vascular insufficiency and, resultantly, controlling her hemichorea.

Anti‐NMDA Receptor Encephalitis with a Favorable Prognosis Despite Delayed Treatment Due to Longstanding Psychiatric Symptoms

Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis can exhibit a wide spectrum of clinical features, including psychiatric symptoms, movement disorders, and autonomic dysfunction. We report an unusual presentation of antiNMDAR encephalitis in a 31-year-old woman. The patient had no preceding flu-like symptom and no history of psychotic episode or drug before symptom onset. She had experienced visual and auditory hallucinations 1 year preceding admission. Her auditory hallucinations were about a strange old woman who forced her to repeat what she said. After 6 months from onset, she had taken risperidone 8 mg at a local clinic because of suspicion of schizophrenia. Her auditory hallucination improved, but dystonia of both arms and catatonia gradually developed 3 months after risperidone treatment. Risperidone was stopped because these features were considered extrapyramidal symptoms induced by risperidone. Nevertheless, the patient’s dystonia worsened over time, despite stopping risperidone treatment. After admission, the oculogyric crisis, dystonia, gait disturbance, tachycardia, and hypersalivation gradually developed and severe dysphagia caused aspiration pneumonia resulting in respiratory failure. Upon neurological examination, the patient showed rigidity and dystonia of all extremities, decreased awareness, and speech disturbance. She was then transferred to the intensive care unit. Brain T2-weighted MRI showed increased signal intensities in the bilateral caudate nucleus, medial temporal lobe, and right insula (Fig. 1). EEG showed diffuse slow activity. Cerebrospinal fluid (CSF) analysis showed white blood cell 3/lL, red blood cell 0/lL, protein 21.7 mg/dL, glucose 78 mg/dL, and unmatched oligoclonal bands. Viral antibody (Ab) tests of CSF were negative. Serum ceruloplasmin and copper levels, 24-hour urine copper level, serum tumor markers (alpha-fetoprotein, carbohydrate-antigen 19-9, and b2 microglobulin), thyroid function test, and serum parathyroid hormone level were within normal limits. Anti-cardiolipin Ab, anti-double-stranded DNA Ab, anti-nuclear Ab, anti-Ro/SSA and anti-La/SSB Ab, and rheumatoid factors were all negative. There was no evidence of underlying malignancy. The patient was treated with baclofen 10 mg three times daily (TID) and clonazepam 0.5 mg TID for dystonia, but no clinical improvement was observed. Antibiotics for aspiration pneumonia and prednisolone (30 mg/day) were administered (1 year after symptom onset). Her serum and CSF was sent to Dr. Dalmau’s laboratory (Department of Neurology, Hospital Clinic, University of Barcelona, Barcelona, Spain) to confirm anti-NMDAR encephalitis, and anti-NMDAR Ab in serum and CSF was positive. Intravenous (IV) steroid (methylprednisolone 1,000 mg) was administered for 3 days, and then IV immunoglobulin was administered for 5 days with 30 mg of oral prednisolone. In addition, plasmapheresis was performed every other day for 10 days, and cyclophosphamide (750 mg/m) was administered 3 times every 2 weeks. The patient was then Figure 1 The patient’s initial brain MRI. The fluid-attenuated inversion recovery images (A–C) show hyperintensity, with no enhancement lesion on T1-weighted images (D–I) in the bilateral medial temporal lobes, basal ganglia, right insula, and periventricular areas.