Seizures Related to Vitamin B6 Deficiency in Adults.

Vitamin B6 is closely associated with functions of the nervous, immune, and endocrine systems. Its deficiency may result in neurological disorders including convulsions and epileptic encephalopathy. Until today, this has only been reported in infants, children, and critically ill adult patients.

Atrophy of the Cerebellar Vermis in Essential Tremor: Segmental Volumetric MRI Analysis.

Postmortem studies of essential tremor (ET) have demonstrated the presence of degenerative changes in the cerebellum, and imaging studies have examined related structural changes in the brain. However, their results have not been completely consistent and the number of imaging studies has been limited. We aimed to study cerebellar involvement in ET using MRI segmental volumetric analysis.

Recurrent seizures following focal motor status epilepticus in a patient with non-ketotic hyperglycemia and acute cerebral infarction.

Focal motor status epilepticus (FMSE) is often associated non-ketotic hyperglycemia (NKH). There are no previous reports describing FMSE with NKH that was accompanied by an acute cerebral infarction and its long term follow-up result. We describe the case of a patient having focal motor status epilepticus (FMSE) associated with non-ketotic hyperglycemia (NKH) and acute cerebral infarction who later developed recurrent unprovoked seizures.

Hashimoto's encephalopathy: South Korean experiences.

Hashimoto's encephalopathy (HE) is a rare neurological disorder. Early diagnosis and treatment are critical to prevent irreversible brain damage. In the present study, we aimed to describe and classify HE on the basis of clinical, neuroimaging, and EEG findings.

Short and long term outcome of bilateral pallidal stimulation in chorea-acanthocytosis.

Background: Chorea-acanthocytosis (ChAc) is a neuroacanthocytosis syndrome presenting with severe movement disorders poorly responsive to drug therapy. Case reports suggest that bilateral deep brain stimulation (DBS) of the ventro-postero-lateral internal globus pallidus (GPi) may benefit these patients. To explore this issue, the present multicentre (n=12) retrospective study collected the short and long term outcome of 15 patients who underwent DBS.

Decreased Metabolism in the Cerebral Cortex in Early-Stage Huntington's Disease: A Possible Biomarker of Disease Progression?

Background And Purpose: Huntington's disease (HD) is an autosomal-dominant inherited neurodegenerative disorder. Genetic analysis of abnormal CAG expansion in the IT15 gene allows disease confirmation even in the preclinical stage. However, because there is no treatment to cure or delay the progression of this disease, monitoring of biological markers that predict progression is warranted.

Nonmotor symptoms in drug-induced parkinsonism and drug-naïve Parkinson disease.

Background: The clinical manifestations of drug-induced parkinsonism (DIP) and Parkinson disease (PD) are nearly indistinguishable, making it difficult to differentiate DIP from PD, especially in the early stages. We compared non-motor symptoms between patients with DIP and those with drug-naïve PD in the early stages using the Non Motor Symptoms Scale (NMSS).

Methods: We prospectively enrolled 28 patients with DIP, 35 patients with drug-naïve PD, and 32 controls with no history of neurological diseases or related medical problems.

Caregiver burden in Parkinson disease with dementia compared to Alzheimer disease in Korea.

We compared caregiver burden in Parkinson disease with dementia (PDD) to that in Alzheimer disease (AD) and examined the factors contributing to the burden in PDD. Totally, 42 patients with PDD and 109 patients with AD and their caregivers participated in this study. The caregiver burden was measured using the Burden Interview (BI).

Globus pallidus interna deep brain stimulation improves chorea and functional status in a patient with chorea-acanthocytosis.

We report a 39-year-old woman with chorea-acanthocytosis (ChAc) who was referred with refractory hyperkinetic movement and truncal bending spasm. She was diagnosed with ChAc with clinical features and laboratory findings of acanthocytosis in peripheral blood smear, and genetic studies revealed novel mutations in the VPS13 gene. Because her symptoms did not respond well to medical treatment, she was in a totally dependent state.

Preliminary study of intravenous amantadine treatment for ataxia management in patients with probable multiple system atrophy with predominant cerebellar ataxia.

Background And Purpose: Multiple system atrophy with predominant cerebellar ataxia is a disabling neurologic disease. However, effective management has not yet been established. We conducted a short-term, open-label preliminary study to assess the benefits of intravenous amantadine treatment in patients with probable multiple system atrophy with predominant cerebellar ataxia.

Factors contributing to spousal and offspring caregiver burden in Parkinson's disease.

Background/aims: Parkinson's disease (PD) is a common neurodegenerative disease with a chronic disease course. The increase in life expectancy of humans worldwide is expected to increase the prevalence and duration of PD; therefore, it is important to determine factors that contribute to the caregiver burden for both clinical and social reasons.

Methods: We surveyed 91 main caregivers of patients, and compared factors contributing to caregiver burden between 50 spouses and 41 offspring of patients.

A Korean Parkinson's disease family with the LRRK2 p.Tyr1699Cys mutation showing clinical heterogeneity.

Background: Although leucine-rich repeat kinase 2 (LRRK2) is the gene most commonly linked to autosomal dominant inherited Parkinson's disease (PD), there have been few reports in Asia, probably because of population-specific differences in allele frequencies.

Methods: We identified a large Korean PD family with the p.Tyr1699Cys mutation in LRRK2 and analyzed genealogical, clinical, and genetic data from the family.

Perioperative stroke in the brain and spinal cord following an induced hypotension.

A 49-year-old woman presented with stupor and paraplegia following an induced hypotension. The temporal relationship to the induced hypotension and the absence of a clear embolic source on diagnostic tests support a causal association between the hypotensive episode and the ischemic infarct. However, despite the association, a cause-and-effect relationship could not be automatically inferred.