An Autopsy Case of Amyotrophic Lateral Sclerosis with Neuroinflammatory change

Neuroinflammation such as reactive gliosis and microglial activations are important pathological findings of ALS. We present a first autopsy case of ALS in Korea related with neuroinflammatory change. A 67-year-old ALS patient suddenly expired due to accidental head trauma. Gross autopsy finding showed marked atrophic change in spinal cord. Pathological finding include a marked loss of motor neurons, reactive gliosis and microglial infiltrations. These findings suggest neuroinflammation may play a role in pathogenesis of ALS.

Current Status of the Diagnosis and Management of Amyotrophic Lateral Sclerosis in Korea: A Multi-Center Cross-Sectional Study

BACKGROUND AND PURPOSE: Recently published, evidence-based guidelines should alter the management of amyotrophic lateral sclerosis (ALS)/motor neuron disease (MND). However, the newest recommendations for ALS/MND therapy are not reflected in actual clinical practice. We sought to evaluate the current status of the diagnosis and management of ALS in Korea. METHODS: The Korean ALS/MND research group was organized in 2010, involving more than 50 neurologists from neuromuscular centers in Korea. Participating centers collected data from April to September 2010 on the diagnosis and management of patients with ALS. Data forms from the ALS patient care database, which is a component of the ALS clinical assessment, research, and education program (http://www.outcomes-umassmed.org/ALS/), were modified and used for data collection. RESULTS: In total, 373 sporadic ALS cases from 35 centers were enrolled. The demographic features and clinical findings were similar to those in previous reports from other countries. The mean age at onset was 50-60 years, and a slight male predominance was observed. The enrolled patients predominantly showed focal onset of cervical or lumbosacral symptoms. Only about one-half of the indicated patients (31.4%) received a physician's recommendation for a parenteral gastrostomy, and 18.1% underwent the procedure. Noninvasive ventilation was recommended in 23% of patients, but applied in only 9.5% of them. Tracheostomy was performed in 12.7% of patients. CONCLUSIONS: The demographic and clinical features of the diagnosis and management of ALS in Korea are similar to those reported in other countries; however, supportive management, as recommended in evidence-based guidelines, are not yet widely recommended or performed for patients with ALS in Korea.

Serial Follow-up of White Matter Connectivity in a Patient with Progressive Multifocal Leukoencephalopathy Presenting Clinical Improvement

Progressive multifocal leukoencephalopathy (PML) is a fatal disease that is characterized by progressive demyelination of the cerebral white matter due to JC viral infection. We report serial magnetic resonance spectroscopy (MRS) and tractography changes in a patient with PML. After a three-cycle treatment with cidofovir and cytarabine, the patient exhibited remarkable improvements in motor and cognitive functions. Follow-up MRS and tractography revealed regeneration of the white-matter fibers. This is the first consecutive neuroimaging study showing improvements in neurological symptoms using MRS and tractography.

Amyotrophic Lateral Sclerosis in Korea: Clinical Characteristics and Prognostic Factors

BACKGROUND: No prospective analyses of clinical characteristics and prognostic factors on amyotrophic lateral sclerosis (ALS) have been performed in Korea. METHODS: The prognosis of 227 sporadic ALS patient with El-Escorial-compatible criteria were evaluated using the serial Korean Amyotrophic Lateral Sclerosis Functional Rating Scale-revised (K-ALSFRS-R), and the progression rate of K-ALSFRS-R (DeltaFS=48-ALSFRS-R at the time of diagnosis). Their clinical characteristics and survival times were also analyzed. The endpoint was defined as either time to death or the tracheostomy state. The effects of individual prognostic factors on disease progression were assessed with the Kaplan-Meier life-table method and multivariate Cox proportional regression analysis. The declining ALSFRS-R curve, epidemiological data, and neuropsychological tests were evaluated. RESULTS: Among 331 motor neuron disease (MND) patients, 227 were finally diagnosed as sporadic ALS. The mean age at onset in 227 sporadic ALS patients was 50.7 years (range 19-76 years). Analysis of sequential ALSFRS-R data revealed that a mean DeltaFS of 0.73/month and a high DeltaFS score were related with early death or a tracheostomy state. Neuropsychological testing revealed nonmotor symptoms, and especially cognitive impairment, had developed in half of our patients. CONCLUSIONS: Our data suggest that DeltaFS is valuable parameter for evaluating prognosis and clinical course of ALS patients, and clinical characteristics obtained from this study would be informative data for understanding epidemiology of ALS in Korea.