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.
Adult ; Arthritis ; Arthritis, Reactive ; Diagnosis, Differential ; HLA-B27 Antigen ; Humans ; Methotrexate ; Nervous System ; Spinal Cord Diseases* ; Spondylarthropathies ; Sulfasalazine ; Urethritis ; Uveitis

Ocular complications of HIV-related cryptococcal meningitis are reasonably common, but complete binocular blindness as the first manifestation of HIV is extremely rare. A 58-year-old man presented with binocular blindness. He experienced blurred vision for 3 days before the blindness. Mild pleocytosis was present in the cerebrospinal fluid, from which Cryptococcus neoformans was cultured. Serology revealed positivity for HIV antibody. He was treated with antifungal and antiretroviral therapy. This case indicates that HIV-related cryptococcal meningitis should be taken into consideration when determining the cause of unexpected sudden binocular blindness.
Blindness* ; Cerebrospinal Fluid ; Cryptococcus neoformans ; HIV ; Humans ; Leukocytosis ; Meningitis, Cryptococcal* ; Middle Aged ; Telescopes*

A small localized infarction in the dorsal pontine area can cause various eye-movement disturbances, such as abducens palsy, horizontal conjugate gaze palsy, internuclear ophthalmoplegia, and one-and-a-half syndrome. However, complete loss of vertical saccades and pursuit with horizontal gaze palsy has not been reported previously in a patient with a small pontine lesion. We report a 67-year-old man with a small dorsal caudal pontine infarct who exhibited total horizontal gaze palsy as well as loss of vertical saccades and pursuit.
Aged ; Humans ; Infarction* ; Ocular Motility Disorders ; Ophthalmoplegia ; Paralysis* ; Saccades*

Idiopathic spinal cord herniation is a rare spinal cord disorder caused by spinal cord prolapse through a adural defect. It is a curable disease, so early detection is of particular importance. We report a 38-year-old woman with Brown-Sequard syndrome which was caused by the thoracic spinal cord herniation. Her weakness was almost completely resolved after surgical management, which emphasizes the importance of early diagnosis and surgical management in this rare disease entity.
Adult ; Brown-Sequard Syndrome* ; Early Diagnosis ; Female ; Humans ; Prolapse ; Rare Diseases ; Spinal Cord Diseases ; Spinal Cord*

Progressive multifocal leukoencephalopathy (PML) is a rare disease that occurs mainly in immunocompromised patients. Despite the progressive nature of the disease, the changes on MRI during the disease course - which may help in monitoring the disease process - have seldom been reported. Here we describe a patient with polymerase-chain-reaction-proven PML examined using serial diffusion-weighted imaging (DWI) and apparent-diffusion-coefficient mapping. Magnetic resonance spectroscopy (MRS) revealed that the demyelinating process was more active without significant neuronal loss at the newer and advancing edge of a lesion than in the older central part of the lesion. This case shows that MRI findings such as DWI and MRS may improve the diagnosis and the understanding of the pathophysiology of PML.
Diagnosis ; Diffusion* ; Humans ; Immunocompromised Host ; Leukoencephalopathy, Progressive Multifocal* ; Magnetic Resonance Imaging ; Magnetic Resonance Spectroscopy* ; Neurons ; Protons* ; Rare Diseases

The dorsolateral medullary syndrome (Wallenberg's syndrome) is produced by infarction of a wedge of lateral medulla posterior to the inferior olivary nucleus, and is usually caused by vertebral artery occlusion. Ipsilateral axial lateropulsion as an initial symptom of vertebral artery occlusion is rare, and the responsible anatomical structure is still uncertain. Here we describe a patient presenting with ipsilateral axial lateropulsion as an initial symptom of vertebral artery occlusion.
Humans ; Infarction* ; Lateral Medullary Syndrome ; Magnetic Resonance Imaging ; Olivary Nucleus ; Vertebral Artery

A 42-year-old man with left posterior inferior cerebellar artery (PICA) infarction presented with akinetic mutism and cognitive impairment. Initially he suffered from akinetic mutism and MRI-documented infarction in the distribution of the left PICA. Twelve days later he developed cognitive impairment. Neuropsychological tests were performed, with the results corrected for age and education being compared with published Korean norms. Impaired performances were evident on executive function testing, with difficulties in planning, abstract reasoning, set-shifting, and perseveration. Akinetic mutism and cognitive-affective syndrome may be a manifestation of unilateral PICA infarction.
Adult ; Akinetic Mutism* ; Arteries ; Education ; Executive Function ; Humans ; Infarction* ; Neuropsychological Tests ; Pica*

Poststroke hypotension may be related to early neurological deterioration and infarct progression by decrease of cerebral perfusion, particularly in patients with ischemic penumbra. However, optimal management guideline of blood pressure in the setting of acute stroke is absent, and remains a matter of debate. We report here a patient who had early neurological deterioration accompanied by systemic hypotension. Phenylephrine-induced hypertensive therapy by imaging-based decision making successfully restored neurologic dysfunction in this patient.
Blood Pressure ; Decision Making ; Humans ; Hypotension ; Neurologic Manifestations ; Perfusion ; Stroke*

BACKGROUND AND PURPOSE: N-methyl-D-aspartate (NMDA)-mediated neurotoxicity and oxidative stress have been implicated in the etiology of amyotrophic lateral sclerosis (ALS). Memantine is a low-affinity, noncompetitive NMDA receptor antagonist that may protect against motor neuron degeneration. METHODS: Thirty transgenic mice expressing the G93A SOD1 mutation were randomly divided into control, low-dose memantine (30 mg/kg/day), and high-dose memantine (90 mg/kg/day) groups, with memantine supplied daily with drinking water beginning at 75 days of age. Body weight, survival, and behavioral performances including a rotarod test, paw grip endurance, and hindlimb extension reflex were assessed in the control and memantine-diet groups. RESULTS: Clinical symptoms were evident in the G93A transgenic mice by 11 weeks of age. Memantine was tolerated well. Compared to control, mice treated with memantine performed better in the rotarod test and hindlimb extension reflex. Moreover, low-dose memantine treatment significantly prolonged the survival of the transgenic mice relative to control mice (141 vs 134 days, p<0.05). CONCLUSIONS: These findings suggest that memantine, even when administered at the time of symptom onset, has beneficial effects on patients with ALS.
Administration, Oral* ; Amyotrophic Lateral Sclerosis* ; Animals ; Body Weight ; Drinking Water ; Hand Strength ; Hindlimb ; Humans ; Memantine* ; Mice ; Mice, Transgenic* ; Motor Neurons ; N-Methylaspartate ; Oxidative Stress ; Reflex ; Rotarod Performance Test

BACKGROUND AND PURPOSE: Zonisamide (ZNS) is a useful antiepileptic drug with a broad therapeutic spectrum. However, there is limited information on the long-term use of ZNS as a monotherapy. This study investigated the long-term effects of ZNS as a monotherapy for the treatment of epilepsy. METHODS: We retrospectively analyzed the records of epilepsy patients treated with ZNS monotherapy at our clinic. We identified outcomes for patients treated with ZNS monotherapy for a minimum of 6 months. Efficacy was quantified as the percentage change in seizure frequency, and safety was assessed by the frequency and types of adverse events. RESULTS: Sixty patients who received ZNS for a minimum of 6 months were included. The mean duration of treatment was 19.8 months (range, 6-37 months), and the mean ZNS dosage was 255 mg/day (range, 100-500 mg/day). Twenty-seven patients (45%) were seizure-free, and an additional 20 patients (33%) had above 50% seizure frequency reduction at the last follow-up visit. Partial seizures with or without secondary generalization and generalized seizures were well controlled by ZNS, whereas complex partial seizures were not. Forty-eight patients (80%) reported mild-to-moderate adverse events, including memory loss (35%), attention deficit (27%), and weight loss (20%). CONCLUSIONS: Long-term ZNS monotherapy is effective at treating a broad spectrum of seizure disorders, except complex partial seizures. However, a specific adverse event, such as cognitive impairment, is common and long-lasting.
Epilepsy* ; Follow-Up Studies ; Generalization (Psychology) ; Humans ; Memory Disorders ; Retrospective Studies ; Seizures ; Weight Loss