Paramagnetic Rims in Multiple Sclerosis and Neuromyelitis Optica Spectrum Disorder: A Quantitative Susceptibility Mapping Study with 3-T MRI

Background@#and Purpose: Iron retained by activated microglia and macrophages in multiple sclerosis (MS) lesions may serve as a marker of innate immune system activation. Among several magnetic resonance imaging (MRI) methods, there has been recent interest in using quantitative susceptibility mapping (QSM) as a potential tool for assessing iron levels in the human brain. This study examined QSM findings in MS and neuromyelitis optica spectrum disorder (NMOSD) lesions obtained with 3-T MRI to assess imaging characteristics related to paramagnetic rims around brain lesions in MS and NMOSD. @*Methods@#This study included 32 MS and 21 seropositive NMOSD patients. MRI images were obtained using two 3-T MRI devices (Ingenia, Philips Healthcare; and Magnetom Verio, Siemens Healthineers) during routine diagnosis and treatment procedures. Multi and single echo gradient echo magnitude and phase images were obtained for QSM reconstruction.QSM images were used to characterize the detected lesions, and the findings were compared between MS and NMOSD. @*Results@#Totals of 71 and 35 MRI scans were performed during the study period in MS and NMOSD patients, respectively. In QSM images, paramagnetic rims were found in 26 (81.2%) MS patients and 1 (4.8%) NMOSD patient. Eight of the 22 MS patients and only 1 of the 10 NMOSD patients who underwent follow-up MRI showed new paramagnetic rims. The paramagnetic rim lesions appeared after enhancement or in new T2-weighted lesions without enhancement. @*Conclusions@#Paramagnetic rims might be a characteristic MRI finding for MS, and therefore they have potential as an imaging marker for differentially diagnosing MS from NMOSD using 3-T MRI.

Neuromyelitis optica spectrum disorder in a patient with spinocerebellar ataxia type 6

@#Spinocerebellar ataxia type 6 (SCA6) is an autosomal dominant, late-onset, slowly progressive cerebellar ataxia due to a pathological CAG repeat expansion in CACNA1A. Inflammation may be involved in the pathogenesis and progression of the trinucleotide repeat expansion disorder. We report a rare case of a 59-year-old woman with SCA6 who developed neuromyelitis optica spectrum disorder (NMOSD). In our case, this combination is coincidental but suggests that an inflammatory response to an unstable CAG repeat may contribute to NMOSD pathogenesis

Ultrasonography in the Early Diagnosis of Diabetic Lumbosacral Radiculoplexus Neuropathy

No abstract available.

Cardiovascular Autonomic Dysfunction in Mild and Advanced Parkinson's Disease

OBJECTIVE: The purpose of the present study was to investigate cardiovascular autonomic dysfunction in patients with Parkinson's disease (PD) with mild to severe stages of motor symptoms and to compare cardiovascular autonomic dysfunction between drug-naïve and dopaminergic drug-treated groups. METHODS: This study included 188 PD patients and 25 age-matched healthy controls who underwent head-up tilt-testing, 24-h ambulatory blood pressure (BP) monitoring and 24-h Holter monitoring. Autonomic function test results were evaluated among groups categorized by motor symptom severities (mild vs. moderate vs. severe) and treatment (drug-naïve or dopaminergic drug treatment). RESULTS: Orthostatic hypotension and supine hypertension were more frequent in patients with PD than in healthy controls. The frequencies of orthostatic hypotension, supine hypertension, nocturnal hypertension and non-dipping were not different among groups. Additionally, no significant differences were detected in supine BP, orthostatic BP change, nighttime BP, nocturnal BP dipping, or heart rate variabilities among groups. CONCLUSIONS: Cardiovascular autonomic dysfunction is not confined to moderate to severe PD patients, and starts early in the course of the disease in a high proportion of PD patients. In addition, dopaminergic drug treatments do not affect cardiovascular autonomic function.

Blood Pressure and Heart Rate Variability in Alzheimer's Disease

BACKGROUND AND PURPOSE: Altered blood pressure (BP) and heart rate variations (HRVs) have been reported in Alzheimer's disease (AD). However, it is unclear how these two manifestations are associated with AD. Therefore, the objective of this study was to investigate BP and heart rate variability in AD compared to that in normal controls, patients with subjective memory impairment (SMI), and patients with mild cognitive impairment (MCI). METHODS: Case-control comparisons were made among AD (n=37), MCI (n=24), SMI (n=17), and controls (n=25). All patients underwent clinical and neuropsychological assessments with 24-h ambulatory BP and Holter monitoring. RESULTS: Patients with AD had higher pulse pressures than those in other groups. In addition, AD patients experienced blunted nocturnal BP dipping associated with declining cognitive status. AD patients also had larger ranges of HRV in parasympathetic domains compared to other groups, especially at night. CONCLUSIONS: Our results suggest that diurnal sympathetic and parasympathetic cardiac variability were significantly disturbed in mild cholinesterase-naive AD patients. This may be an indirect sign of disturbed integrity to the sleep-wake cycle in mild AD.

Posterior Reversible Encephalopathy Syndrome as a Simultaneous Manifestation of Guillain-Barre Syndrome in an Elderly Female Patient

We report a 62-year-old woman with a simultaneous occurrence of posterior reversible encephalopathy syndrome (PRES) and Guillain-Barre syndrome (GBS). The simultaneous presence of PRES in GBS is unusual and can easily escape recognition, with potentially serious consequences for the patient. Previous reports showed that the simultaneous or initial manifestation of PRES in GBS is more common in elderly female. The preponderance of female in older age may be due to age and gender related difference in sympathetic nerve activity.

Numb Chin Syndrome with Concomitant Painful Ophthalmoplegia Leading to a Diagnosis of Diffuse Large B Cell Lymphoma / Journal of the Korean Cancer Association, 대한암학회지

Painful ophthalmoplegia (PO) and concomitant numb chin syndrome (NCS) is a very rare event. There are a few reports in the literature about PO and concomitant NCS that have preceded the diagnosis of a malignancy. In this report, we describe a patient with diffuse large B cell lymphoma who presented with PO and concomitant NCS as the initial symptom of the disease.

Crossed Cerebellar Diaschisis in Complex Partial Status Epilepticus

Crossed cerebellar diaschisis has been described mostly in hemispheric stroke and supratentorial tumors with positron-emission tomography (PET) and single photon emission computed tomography (SPECT). Rarely it has been described with brain diffusion-weighted MRI of status epilepticus. We report a patient with status epilepticus, who developed MRI abnormalities in the cerebral cortex and contralateral cerebellum. EEG abnormalities correlated anatomically with the cerebral cortex of image change. An aggressive medication resulted in seizure control, reversal of neurologic deficit, and improvement or resolution of the MRI and EEG abnormalities in 3 weeks. We concluded that both localization and resolution of lesions may be explained by reversible excitotoxic cell damage due to seizure-related excessive synaptic activity.

Crossed Cerebellar Diaschisis in Complex Partial Status Epilepticus / 대한간질학회지

Crossed cerebellar diaschisis has been described mostly in hemispheric stroke and supratentorial tumors with positron-emission tomography (PET) and single photon emission computed tomography (SPECT). Rarely it has been described with brain diffusion-weighted MRI of status epilepticus. We report a patient with status epilepticus, who developed MRI abnormalities in the cerebral cortex and contralateral cerebellum. EEG abnormalities correlated anatomically with the cerebral cortex of image change. An aggressive medication resulted in seizure control, reversal of neurologic deficit, and improvement or resolution of the MRI and EEG abnormalities in 3 weeks. We concluded that both localization and resolution of lesions may be explained by reversible excitotoxic cell damage due to seizure-related excessive synaptic activity.

Spinal Epidural Metastasis Presenting Atypical Images in Hepatocellular Carcinoma

No abstract available.

Toxoplasmosis Showing Atypical Brain Lesion in an Immunocompetent Patient With Complex Partial Seizure

Toxoplasma gondii is a protozoan parasite that typically manifests as multiple brain lesions of varying sizes. Toxoplasmosis usually causes neurological complications in immunocompromised patients while it is mostly asymptomatic in healthy subjects. A 19-year-old Peruvian woman presented with seizure, and MRI of the brain disclosed a nodular lesion in the left parietal lobe. This case of toxoplasmosis in an immunocompetent patient required extensive differential diagnoses and treatments.

Japanese-B Virus Encephalitis With Biphasic Illness Pattern

Japanese-B virus encephalitis (JE), the most common human endemic and epidemic encephalitis, usually has a uniphasic illness with a variable outcome. A 49-year-old woman with cognitive dysfunction from JE had improved over 3 weeks. Brain MRI revealed multiple lesions in the bilateral thalamus, caudate nuclei and medial temporal lobe. Two weeks after discharge, the patient showed re-aggravation of the cognitive dysfunction and lapsed into coma. We report a patient with JE having showed biphasic illness pattern.

Pure Limb Ataxia as Isolated Manifestation in Lateral Medullary Infarction

Pure limb ataxia without other common symptoms of Wallenberg syndrome has rarely been reported as manifestation of lateral medullary infarction. We describe a patient with lateral medullary infarction who presented with almost isolated right limb(mainly lower limb) ataxia without usual symptoms such as hemibody sensory changes, dysphagia, hoarseness, hiccup, and Horner sign. The brain magnetic resonance image showed small ischemic lesion involving the dorsolateral portion of the medulla oblongata.

Pure Sensory Deficit at the T4 Sensory Level as an Isolated Manifestation of Lateral Medullary Infarction

In rare cases restricted sensory deficits along the somatotopic topography of the spinothalamic tract can develop from a lateral medullary infarction. To our knowledge, isolated dermatomal sensory deficit as a single manifestation of a lateral medullary infarction has not been reported previously. A 58-year-old man presenting with sudden left-sided paresthesia complained of sensory deficit of pain and temperature below the left T4 sensory level without other neurologic deficits. Diffuse- and T2-weighted magnetic resonance imaging (MRI) of the brain showed high signal intensities in the right lower medulla oblongata, whereas thoracic-spine MRI and somatosensory evoked potentials produced normal findings.

Hemiballism as First Isolated Manifestation Following Caudate Infarction

Hemiballism is a rare hyperkinetic involuntary movement disorder that presents with unilateral forceful, flinging, large amplitude of proximal limbs. The most consistent neuropathological findings in hemiballism are a lesion of the contralateral subthalamic nucleus and pallidosubthalamic tract. However, we experienced a patient with pure hemiballism as isolated manifestation of acute ischemic stroke without other neurological abnormal symptoms such as chorea or dystonia. Brain magnetic resonance image showed acute ischemic stroke in right caudate nucleus but not subthalamic nucleus.

A Case Report of Reiter's Syndrome with Progressive Myelopathy

Reiter's syndrome belongs to the family of spondyloarthropathies that usually present with a triad of arthritis, urethritis, and uveitis. The diagnostic criteria include clinical, radiological, and genetic findings, and the response to treatment. Nervous system involvement in Reiter's syndrome is extremely rare. We report here on a 36-year-old man who initially presented with progressive cervical myelopathy and was diagnosed as Reiter's syndrome 2 years later. The myelopathy was stable after treatment with methotrexate and sulfasalazine. This case suggests that Reiter's syndrome can present as progressive myelopathy and should be considered in the differential diagnosis of treatable myelopathies.

Two Novel Mutations in the HSN2 Gene Identified in a Korean Patients with Hereditary Sensory Autonomic Neuropathy Type II

A 38-year-old Korean man was diagnosed with hereditary sensory and autonomic neuropathy (HSAN) type 2 because of his chronic sensory neuropathy and progressive acro-mutilation. Genetic analysis revealed that he was a compound heterozygous for two novel mutations in the HSN2 gene including a nonsense mutation (Gln73X) and a 1-bp insertion mutation (Asp379fsX1). To our knowledge, this is the first report of a genetically confirmed case of HSAN type 2 in the Asian population and supports the genetic homogeneity of this rare disease.

Unilateral Epileptic Negative Myoclonus Following Focal Lesion of the Postcentral Cerebral Cortex Due to Acute Middle Cerebral Infarction

Here we report a patient who suffered an acute infarction of the contralateral postcentral cerebral cortex and subsequently developed unilateral partial epilepsy with negative myoclonus. The findings of brain magnetic resonance imaging, polygraphic recordings of the postcentral somatosensory area, and response to anticonvulsant treatment support the presence of a cause-and-effect relationship, thereby providing evidence for a pathophysiological substrate for epileptic negative myoclonus.

Catastrophic Respiratory Failure Associated with Unilateral PICA Infarction Involving Lateral Medullar Region

Medullar respiratory centers are composed of ventral and dorsal groups. A direct infarction to their structure could lead to a complete loss of respiratory drive, despite unilateral brainstem lesion is rarely associated with central respiratory dysfunction. A 70-year-old man was admitted with sudden dizziness and disequilibrium without motor weakness. Brain MRI (diffuse weight image) shows high signals intensities on left PICA territory of cerebellum and medulla oblongata including reticular formation, nucleus of tractus solitarius, nucleus ambiguus, and nucleus retroambiguus but sparing corticospinal and corticobulbar pathway. On 3rd hospital day, he had a complete loss of respiratory drive involving both autonomic and voluntary components. He didn't get the respiratory drive during CO2 retention while his consciousness and motor power were preserved.

A Patient of Parkinsonism Combined with Frontotemporal Dementia

Frontotemporal dementia as subtype of Frontotemporal lobe dementia is a neurological disorder characterized by personality changes, deteriaoration of memory and executive functions as well as stereotypical behaviour. Parkinsonism was sometimes associated with other degenerative disease, such as Alzheimer disease. However, Parkinsonism accompanied with frontotemoporal lobe dementia had hardly been described. We report a 68 year-old man with pakinsonian symptoms associated with frontotemporal dementia. He complainted personality change, other cognitive disorder with sparing memory function, bradykinesia, postural instability, stooped posture, and gait disturbance.