Relation of Bony Carotid Canal Diameter and Clinical Manifestations in Patients with Moyamoya Disease / 대한임상신경생리학회지

BACKGROUND: Moyamoya disease is characterized by a progressive stenosis or occlusion of the intracranial internal carotid artery and/or the proximal portion of the anterior cerebral artery and middle cerebral artery. Whether the onset time was childhood or adulthood, the bony carotid canal diameter might be different, but reflects the size of internal carotid artery passing through the bony carotid canal. In this study, we aimed to identify the relationship between bony carotid canal diameter and clinical manifestation. METHODS: 146 consecutive patients diagnosed with moyamoya disease by brain imaging studies were included. We measured the diameter of a transverse portion of bony carotid canal on bone window of a brain computed tomography(CT) image. Patients were divided into two groups, ischemic or hemorrhagic stroke according to clinical manifestation. As a result, 115 patients were included. The Suzuki stage was used as criteria for disease progression. RESULTS: Bony carotid canal diameter was 3.6 ± 0.5 (right) and 3.6 ± 0.4 (left) in the hemorrhagic stroke group, and 3.7 ± 0.4 (right) and 3.6 ± 0.4 (left) in the ischemic stroke group. The bony carotid canal diameter of the moyamoya vessels (3.6 mm) was smaller than the diameter of non-moyamoya vessels (3.8 mm), significantly (p= 0.042). However, there was no difference in the collateral patterns and clinical manifestation in a comparison of both groups. CONCLUSIONS: In our study, there was no significant difference of clinical manifestations and collateral patterns depend on the bony carotid canal diameter in patients with moyamoya disease. These findings suggest that the clinical presentations of moyamoya disease are not related to the onset time of the disease.

Vascular Hyperemia and Crossed Cerebellar Diaschisis in MELAS Patient Presented as Stroke-Like Episode and Seizure

Mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) is one of the mitochondrial disorders that can present as a stroke-like episode or seizure. Although the pathophysiology of MELAS remains inconclusive, the main possibilities are thus far thought to be mitochondrial cytopathy and angiopathy. This case report describes a 61-year-old woman diagnosed with MELAS who presented simultaneously with vascular hyperemia and crossed cerebellar diaschisis.

Vascular Hyperemia and Crossed Cerebellar Diaschisis in MELAS Patient Presented as Stroke-Like Episode and Seizure

Mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) is one of the mitochondrial disorders that can present as a stroke-like episode or seizure. Although the pathophysiology of MELAS remains inconclusive, the main possibilities are thus far thought to be mitochondrial cytopathy and angiopathy. This case report describes a 61-year-old woman diagnosed with MELAS who presented simultaneously with vascular hyperemia and crossed cerebellar diaschisis.

Validity and Reliability of a Korean Version of the National Institutes of Health Stroke Scale

BACKGROUND AND PURPOSE: The National Institutes of Health Stroke Scale (NIHSS) is a clinical assessment tool that is widely used in clinical trials and practice to evaluate stroke-related neurological deficits. The aim of this study was to determine the validity and reliability of the Korean version of the NIHSS (K-NIHSS) for evaluating Korean stroke patients. METHODS: The K-NIHSS was translated and adapted with regard to cultural and linguistic peculiarities. To examine its content validity, we quantified the Content Validity Index (CVI), which was rated by 11 stroke experts. The validity of the K-NIHSS was assessed by comparison with the Glasgow Coma Scale (GCS), the modified Rankin Scale (mRS), and the Barthel Index. The reliability of the K-NIHSS was evaluated using the unweighted kappa statistics for multiple raters and an intraclass correlation coefficient (ICC). RESULTS: The CVI of the K-NIHSS reached 0.91-1.00. The median K-NIHSS score at baseline was 3 (interquartile range, 2-7), and the mean+/-SD score was 6.0+/-6.6. The baseline K-NIHSS had a significantly negative correlation with the GCS at baseline and the Barthel Index after 90 days. The K-NIHSS also had a significantly positive correlation with the mRS after 90 days. Facial paresis and dysarthria had moderate interrater reliability (unweighted kappa, 0.41-0.60); kappa values were substantial to excellent (unweighted kappa, >0.60) for all the other items. The ICC for the overall K-NIHSS score was 0.998. The intrarater reliability was acceptable, with a median kappa range of 0.524-1.000. CONCLUSIONS: The K-NIHSS is a valid and reliable tool for assessing neurological deficits in Korean acute stroke patients.

Clinical Characteristics of Acute Ischemic Stroke while Sleep and Awake / 대한뇌졸중학회지

BACKGROUND: Ischemic stroke occurring during sleep is still an unexplored area of cerebrovascular event. As the exact onset time of stroke while sleeping (SWS) cannot be determined, these patients are generally excluded from the thrombolytic therapy of acute ischemic stroke. The aim of this study was to know whether differences in clinical features exist between patients suffering a SWS and those with stroke while awake (SWA). METHODS: We reviewed the medical records of acute ischemic stroke patients consecutively registered in Hallym Stroke Databank between January 1999 and June 2007. We compared the risk factors and clinical features between the SWS and SWA groups. RESULTS: A total of 2,962 patients were included in the study, of which 821 (27.7%) were SWS. No differences between SWS and SWA were identified with regard to baseline clinical characteristics and risk factors except a history of smoking. In stroke subtype, small vessel occlusions were more frequently in SWS group than SWA group. Intravenous rt-PA treatments were performed frequently in the SWA group. Clinical outcomes at discharge were better in SWA group than SWS group. CONCLUSION: This study suggest that no major differences were exist in clinical characteristics between SWS and SWA patients, except the history of smoking. Clinical outcomes of patients with ischemic stroke within 6 hours after stroke onset were poor in SWS group. In SWS group, relatively little chances of thrombolysis might be the explanation of these finding.

A Case of Purpura Associated with Levetiracetam / 대한간질학회지

Levetiracetam has not been reported to cause purpura. We report a case of purpura associated with levetiracetam. A 81-year-old man suffered from post-stroke seizure. Seizure was well controlled with valproate. Valproate was discontinued due to pancytopenia and levetiracetam was prescribed when he was admitted with pneumonia. A few days later, he recovered from pancytopenia and pneumonia. However, he was rehospitalized due to multiple variable sized-purpura on the whole body surface without thrombocytopenia. After discontinuing levetiracetam, purpura disappeared.

Reversible Abducens Nerve Palsy Following Transvenous Embolization of Cavernous Sinus Dural Arteriovenous Fistula

Transvenous embolization has become the treatment of choice for cavernous sinus dural arteriovenous fistula(cDAVF). However, there are potential complications associated with this procedure such as cranial nerve palsies and venous perforations. A 66-year-old woman presented with a 2-week left periorbital swelling and conjunctival injection. Brain MRI showed engorgement of the left superior ophthalmic vein. Cerebral angiography revealed a dural arteriovenous shunt of the cavernous sinus with retrograde venous drainage into the superior ophthalmic vein. Her proptosis and conjunctival injection resolved completely after transvenous embolization of cDAVF. However, an abducens nerve palsy developed the day after the procedure, which, fortunately, resolved spontaneously. She was symptom-free at the follow- up evaluation 2 months later. The abducens nerve palsy related to the transvenous embolization of cDAVF was presu- med due to either dense packing of the sinus, venous thrombosis, or direct nerve injury. We report a case of transient abducens nerve palsy associated with transvenous embolization of cDAVF, suggesting the benign course of this com- plication.

Inherited metabolic diseases in the urine organic acid analysis of complex febrile seizure patients / 소아과

PURPOSE: Seizure associated with fever may indicate the presence of underlying inherited metabolic diseases. The present study was performed to investigate the presence of underlying metabolic diseases in patients with complex febrile seizures, using analyses of urine organic acids. METHODS: We retrospectively analyzed and compared the results of urine organic acid analysis with routine laboratory findings in 278 patients referred for complex febrile seizure. RESULTS: Of 278 patients, 132 had no abnormal laboratory findings, and 146 patients had at least one of the following abnormal laboratory findings: acidosis (n=58), hyperammonemia (n=55), hypoglycemia (n=21), ketosis (n=12). Twenty-six (19.7%) of the 132 patients with no abnormal findings and 104 (71.2%) of the 146 patients with statistically significant abnormalities showed abnormalities on the organic acid analysis (P<0.05). Mitochondrial respiratory chain disorders (n=23) were the most common diseases found in the normal routine laboratory group, followed by PDH deficiency (n=2 ) and ketolytic defect (n=1). In the abnormal routine laboratory group, mitochondrial respiratory chain disorder (n=29) was the most common disease, followed by ketolytic defects (n=27), PDH deficiency (n=9), glutaric aciduria type II (n=9), 3-methylglutaconic aciduria type III (n=6), biotinidase deficiency (n=5), propionic acidemia (n=4), methylmalonic acidemia (n=2), 3-hydroxyisobutyric aciduria (n=2), orotic aciduria (n=2), fatty acid oxidation disorders (n=2), 2-methylbranched chain acyl CoA dehydrogenase deficiency (n=2), 3-methylglutaconic aciduria type I (n=1), maple syrup urine disease (n=1), isovaleric acidemia (n=1), HMG-CoA lyase deficiency (n=1), L-2-hydroxyglutaric aciduria (n=1), and pyruvate carboxylase deficiency (n=1). CONCLUSION: These findings suggest that urine organic acid analysis should be performed in all patients with complex febrile seizure and other risk factors for early detection of inherited metabolic diseases.

A Case of Oxcarbazepine Induced Beau's Line / 대한간질학회지

Beau's line is a grooved transverse line on the fingernail or toenail, and is considered as a kind of nail disorder, It is known to occur with temporary arrest of nail matrix proliferation. The causes of Beau's line comprise severe systemic illness as well as drug ingestion. A 51-year-old male patient had developed Beau's line on all his fingers after about three months of oxcarbazepine administration. These nail changes disappeared spontaneously with nail growth two months later. The authors report a case of oxcarbazepine induced Beau's line.

Middle Cerebral Artery Infarction Associated with Osler-Weber-Rendu Disease

Osler-Weber-Rendu disease is characterized by epistaxis, cutaneous telangiectasia, and visceral arteriovenous malformations (AVMs). A 65-year-old male with recurrent epistaxis, old infarction and recent multiple rib fractures caused by a bicycle accident had middle cerebral thromboembolic infarctions when he stopped taking clopidogrel due to massive epistaxis and hemopneumothorax. On examinations, there was no focal cerebral stenosis, but there were telangiectasia, pulmonary and hepatic AVMs. A suspicious Osler-Weber-Rendu disease patient should be evaluated by proper screening and regular follow-up to minimize serious sequelae such as thromboembolic stroke.

A Case of Recurrent Hepatic Encephalopathy Secondary to Spontaneous Intrahepatic Portosystemic Venous Shunt

The portosystemic venous shunts within the hepatic parenchyma, a rare disease can lead to hepatic encephalopathy. The etiology of intrahepatic portosystemic venous shunt may be either congenital or acquired such as secondary to portal hypertension due to chronic liver damage. A 61-year-old women was admitted to our hospital with recurrent encephalopathy. Liver function tests, abdominal computerized tomography, ultrasound and arterial portography revealed an intrahepatic portosystemic venous shunt in a noncirrhotic liver. Thus, recurrent encephalopathy should be closely evaluated for detection of rare causes.

Relationship between Homocysteine Level, White Matter Lesion and Cognitive Decline

BACKGROUND: Hyperhomocysteinemia increase the risk of vascular disease, dementia and cognitive impairment. The effect of homocysteine on cognitive function may be as a consequence of direct neurotoxicity or indirectly through cerebrovascular disease. Thus we examined the association between homocysteine levels and cognitive function, and assess whether this may be mediated by cerebral white matter lesions. METHODS: Non-dementic participants were recruited from March to December 2005, and underwent brain MRI. White matter hyperintensities(WMH) were rated visually by 4-class WMH grades. Informations of age, gender, education and vascular risk factor were obtained from interview. Cognitive function were assessed by using K-MMSE and HDS-R. The cross-sectional relationship between homocysteine level and cognitive test score was assessed by multiple regression analysis. RESULTS: Severe WMH groups more exposed to vascular risk factor such as age, hypertension and higher levels of homocyteine. More severe WMH grades also had lower K-MMSE and HDS-R score. Higher group of homocysteine concentration were significantly related to poorer performances on K-MMSE and HDS-R tests. In multiple linear regression analyses for the relationship between homocysteine and cognitive performances, higher homocysteine level was also related to lower cognitive performances. Adding cerebral WMH changes to the level of homocysteine did not significantly change the effect of homocysteine on cognitive ability, and cerebral WMH had a separate and additive effect with homocysteine. CONCLUSIONS: Elevated homocysteine level is associated decreased cognitive score and cerebral white matter lesion on MRI is independent association to the cognitive abilities.

The Abnormalities of Retinal Arteriole in Atherothrombotic Ischemic Stroke Patients Representing the Changes of Cerebral Vasculature Indirectly

PURPOSE: Retinal blood vessels and cerebral small vessels possess similar characteristics anatomically, physiologically and embryologically. We studied the availability of abnormal fundus findings of patients who had an atherothrombotic ischemic stroke and who have the risk factors. METHODS: Fundus photographs and brain images were taken in patients who had a first-ever symptomatic ischemic stroke of large artery atherosclerosis (LAA) or small vessel occlusion (SVO) from March 2004 to February 2005. We analyzed the association between fundus abnormalities and ischemic stroke subtypes. RESULTS: Based on brain MRI and MRA, a total of 47 patients were classified into SVO and LAA groups. The SVO group consisted of 27 patients (mean age: 69.7 years), and the LAA group consisted of 20 patients (mean age: 65.4 years). The control group comprised 15 patients (mean age: 64.9 years). The baseline characteristics were similar among the three groups. The severity of the retinal arteriolar narrowing and sclerosis were associated with hypertension. Compared to the control group, both the SVO and LAA groups showed more severe arteriolar sclerosis, the SVO group showed more severe arteriolar narrowing and the LAA group showed more frequent AV crossing and retinal exudate. CONCLUSIONS: Retinal arteriolar abnormalities such as arteriolar narrowing and sclerosis are more severe in atherothrombotic ischemic stroke patients. Indirectly, retinal microvascular changes may indicate the status of the cerebral vasculature. Thus, analysis of fundus findings is useful for predicting an atherothrombotic ischemic stroke and planning follow-up examinations.

Is the Intensive Anticonvulsant Treatment for Control of Acute Posthypoxic Myoclonic Status Epilepticus Necessary?

BACKGROUND: Acute posthypoxic myoclonic seizure is not an uncommon presentation in clinical practices. However, there have been only a few reports which include detailed description on the treatment of acute posthypoxic myoclonic status. METHODS: We retrospectively analyzed the etiology, clinical course, nature and duration of myoclonus after hypoxic brain damage during a ten year period from 1992 to 2002. Patients who had possible explanations for myoclonic seizure other than hypoxic-ischemic events were excluded. RESULTS: Eighty-two patients aged 24 to 81 years old were included in this study. All but 3 patients with pulmonary edema had a history of cardiorespiratory arrest caused by probable cardiogenic origin, acute asthma attack and upper airway obstruction. Ictal EEGs demonstrated intermittent generalized spike and polyspike activities or biPLEDs. Most patients died within a week and 6 patients remained in persistent vegetative states. The clinical outcome was not affected by the drug response of myoclonic status epilepticus or etiology of hypoxia. CONCLUSIONS: Posthypoxic myoclonic status reflects severe and diffuse cortical damage, and implies a very poor prognosis. It may be a transient manifestation of severe cortical damage before massive and irreversible neocortical neuronal death, and aggressive anticonvulsant drug treatment would not be necessary in most patients.

A Case of Encephalitis with a Reversible Splenial Lesion on a Diffusion Weighted MRI Image

Reversible splenial lesion may occur in encephalitis rarely. A 21-year-old man was admitted with viral encephalitis. High signal on the diffusion MRI and low signal on the ADC map image was showed in the splenium. As the patient improved, the lesion suggestive of cytotoxic edema was completely resolved one month later. Although the concrete pathophysiology of focal cytotoxic edema in encephalitis is unclear yet, MRI is valuable diagnostic tool to manage encephalitis in the view of prognosis.

Status Epilepticus after Mye lography with Inadve rtent Injection of Diatrizoate

A 37-year-old male patient was transferred from a local orthopedic clinic due to status epilepticus following a myelography with diatrizoate. On his laboratory findings, metabolic acidosis was prominent. Non-enhanced brain CT scan showed a high densinty in the subarchnoid space. With careful respiratory support, hydration, correction of acid-base balance, administration of dexamethasone, and anticonvulsant therapy, he completely recovered after 3 days. Diatrizoate is an ionic, iodinated and hyperosmolar X-ray contrast medium. It must not be injected intrathecally because of the risk to the central nervous system. The possible mechanisms are anaphylactic reaction due to iodine hypersensitivity, hyperosmolar effect, and direct chemotoxicity by ionicity and chemical structure itself.