A Case of Anterior Communicating Artery Aneurysm Impending Rupture Presenting with Isolated Painful Adduction Palsy Mimicking Ischemic Internuclear Ophthalmoplegia

Anterior communicating artery (ACoA) aneurysms may rarely lead to oculomotor nerve palsy. We present here interesting cases in which isolated unilateral adduction paresis mimicking internuclear ophthalmoplegia (INO) was one of the symptoms of suspicious impending ruptured aneurysm of the ACoA. Careful neurologic examination is crucial for early discrimination with INO and oculomotor palsy.

Oculomotor Nerve Palsy that Developed after Endovascular Treatment in an Acute Stroke Patient with a Dissecting Occlusion of the Extracranial Internal Carotid Artery

Oculomotor nerve palsy is underrecognized clinical manifestation of extracranial internal carotid artery (ICA) dissection. Herein we report a case of complete oculomotor nerve palsy following endovascular revascularization, which developed in a patient with acute stroke due to extracranial ICA dissecting occlusion. We also discuss about the development mechanism of oculomotor nerve palsy, considering the vascular anatomy and the possibility of periprocedural complications during endovascular treatment.

Venous Thromboembolism Following Acute Ischemic Stroke: A Prospective Incidence Study

BACKGROUND: Asians were known to have a relatively lower incidence of venous thromboembolism (VTE), and there is insufficient evidence to suggest a specific D-dimer threshold level for screening VTE in patients with acute stroke. METHODS: We prospectively enrolled patients with acute ischemic stroke admitted to Jeju National University Hospital. The inclusion criteria were: 1) aged ≥18 years, 2) admission within seven days of symptom onset, and 3) an initial National Institute of Health Stroke Scale (NIHSS) score >1 for the affected lower limb. Ultrasound scans of the lower limbs and plasma D-dimer assays were performed on days 7–14 and 15–28 after stroke onset. RESULTS: Of 285 patients admitted during the study period, 52 patients met inclusion criteria (mean age 74.5, male 40.4%, median initial NIHSS score 12, and unable to walk unassisted at discharge 76.9%). During 7–14 days, 23 of 52 patients (44.2%) had a D-dimer level above 1.57 mg/L, and 9.6% had a level above 5.50 mg/L. Proximal deep vein thrombosis (DVT) was detected in 3 patients (5.8%, 95% confidence Interval 1.2–16.0%) on ultrasound examination. All DVTs were found in elderly female patients with severe leg weakness. No patient was diagnosed with pulmonary embolism during the study period. CONCLUSION: The incidence of VTE seems to be very low among Korean patients with acute ischemic stroke. Advanced age, female sex, and severe leg weakness were important risk factors for developing DVT in this study.

Sinking Skin Flap Syndrome after Decompressive Craniotomy

Sinking skin-flap syndrome is a rare complication of a skull defect after craniotomy. A 69-year-old woman visited our hospital and was diagnosed with a left middle cerebral artery infarction. Decompressive craniotomy was performed for cerebral herniation on the following day. However, on day 25 of hospitalization her mental status worsened and her scalp appeared sunken. This case suggests that sinking skin-flap syndrome can develop from a skull defect after decompressive craniotomy.

A Case of Rapid Eye Movement Sleep Behavior Disorder during Continuous Positive Airway Pressure Titration in a Patient with Multiple System Atrophy

Sleep disorders are commonly observed in multiple systemic atrophy (MSA). The rapid eye movement (REM) sleep behavior disorder (RBD) is characterized by loss of normal voluntary muscle atonia during REM sleep. It usually presents during early course, and disappears over the course of disease progression. Sleep-disordered breathing (SDB) is also common sleep disorder in MSA which can be life-threatening, and continuous positive airway pressure (CPAP) treatment is useful in these patients. A 74-year-old woman with MSA presented for nocturnal respiratory disturbance. She had a five-year history of dream enacting behaviors, which had disappeared four months prior. Polysomnography revealed frequent stridor and sleep hypopnea. During the following full nigh CPAP titration for SDB, dream enacting behavior was observed during REM sleep stage. In MSA patients with SDB, CPAP administration may lead to increase REM sleep stage. An increase in REM sleep stage, which previously had been deprived, may have trigger RBD symptoms to reappear. The CPAP treatment should be considered with great caution in these patients.

Diagnostic Sensitivity and Specificity of Residual Latency and Terminal Latency Index in the Diagnosis of Carpal Tunnel Syndrome

BACKGROUND: Conventional nerve conduction studies (NCS) are used in the diagnosis of carpal tunnel syndrome (CTS). The median terminal latency index (TLI) and median residual latency (RL) are parameters calculated to identify abnormalities in distal segments of the median motor nerve. The objective of this study was to determine the sensitivity and specificity of TLI and RL together with NCS in the diagnosis of CTS. METHODS: This prospective study involved 83 hands of 47 patients with suspected CTS. Conventional NCS were performed using Oh's method. Control data were obtained from the 68 hands of 40 healthy volunteers. The diagnostic sensitivity and specificity of TLI and RL were calculated and compared with those of conventional NCS. We divided the CTS patients into four groups based on their electrophysiological severity, and compared the TLI and RL values between these groups. RESULTS: TLI and RL were 0.20+/-0.03 (mean+/-SD) and 3.62+/-0.90, respectively, in the patients, while the corresponding values, in the healthy control, were 0.29+/-0.03 and 2.08+/-0.30. The sensitivities of TLI and RL in diagnosing CTS were 75.9% and 86.3%, respectively. Compared with median motor terminal latency, the sensitivities of TLI and RL in diagnosing CTS was found to be higher. Moreover, the diagnostic sensitivities of TLI and RL were significant better for the severe group than for the mild and moderate severity group. CONCLUSIONS: We conclude that measuring TLI and RL of the median nerve may increase the sensitivity in diagnosing CTS and also provide information about its electrophysiological severity.

Diagnostic Sensitivity and Specificity of Residual Latency and Terminal Latency Index in the Diagnosis of Carpal Tunnel Syndrome

BACKGROUND: Conventional nerve conduction studies (NCS) are used in the diagnosis of carpal tunnel syndrome (CTS). The median terminal latency index (TLI) and median residual latency (RL) are parameters calculated to identify abnormalities in distal segments of the median motor nerve. The objective of this study was to determine the sensitivity and specificity of TLI and RL together with NCS in the diagnosis of CTS. METHODS: This prospective study involved 83 hands of 47 patients with suspected CTS. Conventional NCS were performed using Oh's method. Control data were obtained from the 68 hands of 40 healthy volunteers. The diagnostic sensitivity and specificity of TLI and RL were calculated and compared with those of conventional NCS. We divided the CTS patients into four groups based on their electrophysiological severity, and compared the TLI and RL values between these groups. RESULTS: TLI and RL were 0.20+/-0.03 (mean+/-SD) and 3.62+/-0.90, respectively, in the patients, while the corresponding values, in the healthy control, were 0.29+/-0.03 and 2.08+/-0.30. The sensitivities of TLI and RL in diagnosing CTS were 75.9% and 86.3%, respectively. Compared with median motor terminal latency, the sensitivities of TLI and RL in diagnosing CTS was found to be higher. Moreover, the diagnostic sensitivities of TLI and RL were significant better for the severe group than for the mild and moderate severity group. CONCLUSIONS: We conclude that measuring TLI and RL of the median nerve may increase the sensitivity in diagnosing CTS and also provide information about its electrophysiological severity.

Takotsubo Cardiomyopathy Associated with Guillain-Barre Syndrome / 대한임상신경생리학회지

A 69-year-old woman presented with a progressive limb weakness. Both clinical and neurophysiological findings were consistent with diagnosis of Guillain-Barre syndrome (GBS). Two days after admission, the patient suffered from an acute coronary syndrome without stenosis at coronary arteriography. Echocardiography revealed left ventricular inferior wall and apical akinesia and decreased ejection fraction. A diagnosis of Takotsubo cardiomyopathy was then made. Left ventricular dysfunction and electrocardiography normalized within one month. Takotsubo cardiomyopathy can be developed as a complication of GBS.

The Association Between Hypertension and Cerebral Microbleeds in Patients With CADASIL

BACKGROUND: Cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an inherited microangiopathy that is caused by mutations in the Notch3 gene. Typical findings from magnetic resonance imaging (MRI) include multiple subcortical lacunae, extensive white-matter change, and multiple cerebral microbleeds (CMBs). CMBs are indicative of bleeding-prone microangiopathy. The rate of intracerebral hemorrhage in CADASIL is higher in Asian patients than in Caucasian patients. However, CMBs have not been thoroughly evaluated in Asian patients. We performed a detailed analysis of the frequency and distribution pattern of CMBs and assessed whether vascular risk factors exert an independent effect on CMBs in Asian CADASIL patients. METHODS: The study population comprised 60 patients who underwent brain MRI, including T2*-weighted gradient-echo sequences. Demographic factors, vascular risk factors, and MRI findings were compared between CADASIL patients with and without CMBs. The impact of vascular risk factors on CMBs, lacunae, and white-matter hyperintensities (WMHs) was assessed by logistic regression analysis. RESULTS: CMBs, which were detected in 34 (56.7%) patients, exhibited a significant predilection for the thalamus (46.7%), subcortical-cortical region (35.0%), and basal ganglia (31.7%). Hypertension, lacunae, and white-matter lesions were more common in patients with CMBs. Hypertension was an independent risk factor for CMBs, lacunae, and WMHs in patients with CADASIL. CONCLUSIONS: This study found that CMBs tended to occur in hypertensive patients with CADASIL. Further studies should focus on elucidating the association between reduced blood pressure and the number of CMBs.

Congenital External Carotid-Internal Carotid Artery Anastomosis

Congenital anomalies of the cervical internal carotid artery (ICA) include anomalous origin, hypoplasia or aplasia, and anomalous branches. Among them, congenital anastomosis of the external carotid artery with the cervical ICA is extremely rare. We report herein two patients with congenital external-internal carotid artery anastomoses at the cervical segment with a remnant of the ICA stump or hypoplasia of the ICA.

Isolated Axial Lateropulsion in Lateral Medullary Infarction

The Wallenberg's syndrome is produced by infarction of lateral medulla. Isolated ipsilateral axial lateropulsion without other common symptoms of Wallenberg syndrome has rarely been reported as manifestation of lateral medullary infarction. The responsible anatomical structure of ipsilateral axial lateropulsion is still uncertain. We describe a patient with lateral medullary infarction who present with isolated ipsilateral axial lateropulsion without other symptoms of Wallenberg syndrome.

Topographical Distribution of Lacunes and Cerebral Microbleeds in CADASIL Affected by Hypertension

BACKGROUND: Cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an inherited small vessel disease caused by mutations in the Notch3 gene. Lacunes may reflect occlusive type microangiopathy. However, cerebral microbleeds (CMBs) may reflect bleeding-prone microangiopathy. In the present study, we aimed to determine whether hypertension influence the distribution and severity of lacunes and CMBs in patients with CADASIL. METHODS: The study population comprised 85 patients who underwent brain MRI, including T1-weighted image, susceptibility weighted image (SWI), and fluid attenuated inversion recovery (FLAIR) image. The patients were divided into two groups depending on the presence or absence of hypertension. In the first, demographic factors, and MRI findings were compared between CADASIL patients with and without hypertension. In the second, we undertook a region by region comparison of number of patients with lacunes or CMBs. RESULTS: The hypertensive group showed a higher incidence of CMBs in lobar area (p<0.001) and basal ganglia (p=0.014). CMBs tend to be observed more frequently in the thalamus (p=0.058), brainstem (p=0.057), and cerebellum (p=0.052) in the hypertensive group. However, hypertensive group demonstrated a higher incidence of lacunes just in lobar area (p=0.040). CONCLUSIONS: Our findings suggest that CMBs may be a more sensitive neuroimaging marker of hypertensive arteriopathy in patients with CADASIL.

Reversible Motor Neuropathy Following Disulfiram Therapy

No abstract available.

Bilateral Paramedian Thalamic Infarction due to Occlusion of Artery of Percheron

The thalamic vascular supply is categorized into the anterior, paramedian, inferolateral, and posterior territories. The artery of Percheron (AOP), a solitary trunk that is an uncommon anatomic variant, provides bilateral arterial supply to the paramedian thalami and the rostral midbrain. Occlusion of this artery results in bilateral thalamic and mesencephalic infarctions. The case described herein is of a patient with AOP who presented with the sudden onset of lethargy.

Bilateral Paramedian Thalamic Infarction due to Occlusion of Artery of Percheron

The thalamic vascular supply is categorized into the anterior, paramedian, inferolateral, and posterior territories. The artery of Percheron (AOP), a solitary trunk that is an uncommon anatomic variant, provides bilateral arterial supply to the paramedian thalami and the rostral midbrain. Occlusion of this artery results in bilateral thalamic and mesencephalic infarctions. The case described herein is of a patient with AOP who presented with the sudden onset of lethargy.

Acute Bilateral Internal Carotid Artery Territory Infarction Associated with a Variant of Aortic Arch Branching

Acute bilateral internal carotid artery occlusion (ICAO) is a rare form of ischemic stroke. The possible role of variants of the aortic arch in bilateral ICAO has never been reported. Two patients presented with abrupt coma with quadriplegia. Magnetic resonance images revealed acute ischemic lesions on both hemispheres, except the area supplied by the vertebrobasilar artery. Both patients showed variation of the origin of the brachiocephalic trunk and left common carotid artery. This variation might explain simultaneous bilateral carotid territory infarctions.

An Autopsy Case of Multiple System Atrophy

Multiple-system atrophy (MSA) is an adult-onset, sporadic, progressive neurodegenerative disease. Clinically, the cardinal features include autonomic failure, parkinsonism, cerebellar ataxia, and pyramidal signs in any combination, of which autonomic failure is an integral component in the diagnosis. Pathologically, MSA is characterized by alpha-synuclein-positive glial cytoplasmic inclusions and neuronal loss, predominantly in the basal ganglia, brainstem, cerebellum, and intermediolateral cell columns of the spinal cord. We report the first case of MSA confirmed by autopsy in Korea.

A Novel PANK2 Mutation in a Patient with Atypical Pantothenate-Kinase-Associated Neurodegeneration Presenting with Adult-Onset Parkinsonism

BACKGROUND: Pantothenate-kinase-associated neurodegeneration (PKAN) is an autosomal recessive neurodegenerative disorder that is characterized by progressive extrapyramidal signs, visual loss, and cognitive impairment. PKAN is caused by mutations in the pantothenate kinase gene (PANK2), which is located on chromosome 20p13 and encodes pantothenate kinase, the key regulatory enzyme in coenzyme-A biosynthesis. CASE REPORT: In this report we describe a case of atypical PKAN with a novel PANK2 mutation, presenting with a 10-year history of postural tremor involving both hands. Upon neurological examination, the patient's face was masked and he spoke in a monotonous voice. The patient presented with mild bradykinesia and rigidity that involved all of the extremities. Horizontal saccadic eye movements were slow and fragmented. Brain MRI revealed a typical "eye-of-the-tiger" sign. A mutation analysis revealed three PANK2 mutations: two in exon 3 (Asp 378Gly and Leu385CysfsX13) and one in exon 4 (Arg440Pro). CONCLUSIONS: Parkinsonism is not an unusual presenting symptom in patients with atypical PKAN, and so it is important for physicians to consider PKAN in the differential diagnosis of patients presenting with young-onset parkinsonism.

Chronic Recurrent Cholangitis Induced by Carbamazepine / 대한간질학회지

Carbamazepine (CBZ) is one of the most commonly used antiepileptic agents. With its potent effects against seizure or neuropathic pain, it also has several undesirable adverse events. CBZ has been known to induce hepatotoxicity because the drug is mainly metabolized through hepatic system, and asymptomatic liver enzyme elevation occurs in 5~10% of patients receiving CBZ. There are several cases of symptomatic hepatitis or hepatic necrosis by CBZ, however, reports of chronic cholangitis associated with CBZ medication are rare. Here, we present a case of chronic recurrent cholangitis by CBZ with pathological evidence.

Posterior Thoracic Spinal Cord Artery Infarction

No abstract available.