Rheumatoid meningoencephalitis is a rare, but severe complication of rheumatoid arthritis (RA). A 64-year-old woman with the history of RA presented with abnormal behaviors and memory impairments since stopping methotrexate due to asymptomatic meningitis before 1 month. Brain MRI still demonstrated leptomeningeal enhancement in right fronto-temporal area, compared with the previous. Brain biopsy revealed multiple yellowish suppurative streaks in dura, and microscopically lymphoplasmic infiltrations and fibroid necrosis surrounded by granuloma. Soon after beginning treatment with corticosteroid and methotrexate, her symptoms improved.
Arthritis, Rheumatoid ; Biopsy ; Brain ; Female ; Granuloma ; Humans ; Leiomyoma ; Magnetic Resonance Imaging ; Memory ; Meningitis ; Meningoencephalitis* ; Methotrexate* ; Middle Aged ; Necrosis

We describe here a case of female patient who presented with mild proximal weakness, myalgia, and markedly elevated CPK, which could be ascribed to paraneoplastic necrotizing myopathy in association with ovarian adenocarcinoma. A histologic examination of the vastus lateralis muscle showed necrosis of muscle fibers without inflammatory cell infiltration. Her neurologic symptoms improved following tumor resection and systemic chemotherapy. Paraneoplastic necrotizing myopathy may be a presenting manifestation of malignancy, and early recognition and prompt treatment are crucial for the clinical improvement.
Adenocarcinoma* ; Drug Therapy ; Female ; Humans ; Muscular Diseases* ; Myalgia ; Necrosis ; Neurologic Manifestations ; Quadriceps Muscle

Epidural blood patch (EBP) is one of the treatments for spontaneous intracranial hypotension (SIH), and its complications have rarely been reported. We report a patient with SIH and developed intracranial hypertension after EBP. The mechanism of rebound intracranial hypertension was uncertain. If the patient presented with a different type of headache or newly developed neurological symptoms after treatment of SIH, rebound intracranial hypertension should be considered and the proper treatment needed quickly.
Blood Patch, Epidural* ; Headache ; Humans ; Intracranial Hypertension* ; Intracranial Hypotension*

Chronic inflammatory demyelinating polyneuropathy (CIDP) has been rarely reported in systemic sclerosis (SSc). We describe the clinical, electrophysiological, and pathologic findings in a 73-year-old female with established limited cutaneous SSc who later developed CIDP. The patient had progressive limb weakness, sensory loss, and slow nerve conduction velocities. Sural nerve biopsy showed evidence of demyelination and remyelination. This case demonstrates that chronic demyelinating polyneuropathy can be an unusual manifestation of SSc, presumably resulting from an immune-mediated process.
Aged ; Autoimmunity ; Biopsy ; Demyelinating Diseases ; Extremities ; Female ; Humans ; Neural Conduction ; Polyneuropathies* ; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating ; Scleroderma, Systemic* ; Sural Nerve

Anti-LGI1 (leucine-rich glioma inactivated-1) antibody encephalitis is one of autoimmune encephalitis. We report a 66-year-old man who presented with frequent, brief dystonic seizures which involve predominantly ipsilateral face and arm without cognitive impairment. Brain MRI showed normal finding. Serum and CSF tests revealed anti-LGI1 antibody. His symptom was not relieved by antiepileptic drugs, but completely controlled after immunotherapy. This case indicates that recognition of the brief, dystonic seizures should do tests for anti-LGI1 antibodies.
Aged ; Antibodies ; Anticonvulsants ; Arm ; Brain ; Encephalitis* ; Glioma ; Humans ; Immunotherapy ; Magnetic Resonance Imaging ; Potassium Channels, Voltage-Gated ; Seizures*

Anti-GQ1 antibody is found in patients with Miller-Fisher syndrome (MFS), atypical MFS, and Bickerstaff's brainstem encephalitis (BBE). These conditions are various manifestations of post-infectious autoimmune disorders, and anti-GQ1b antibodies play a core pathogenic role. So they are referred as the 'anti-GQ1b antibody syndrome'. We report two cases of recurrent anti-GQ1b antibody syndrome.
Antibodies ; Brain Stem ; Encephalitis ; Humans ; Miller Fisher Syndrome ; Recurrence

BACKGROUND: The outcome of spontaneous intracranial hypotension (SIH) is unpredictable and some patients have persistent and often incapacitating symptoms. This study was aimed to investigate whether abnormalities on initial magnetic resonance imaging (MRI) can predict the outcome in patients with SIH. METHODS: We retrospectively included 44 patients with SIH. Brain MRI was available for all patients. Treatment consisted of conservative treatment and/or high-volume epidural blood patching. Patients were divided into two groups: favorable or non-favorable group. Favorable group was defined as clinical improvement by conservative therapy or one trial of autologous epidural blood patching; non-favorable group as more than two week of admission, two or more trials of autologous epidural blood patching, or relapse of orthostatic headache. RESULTS: Twenty-one (48%) of 44 patients were classified as the favorable group. The non-favorable group had several abnormal findings on brain MRI (16 cases vs. 5 cases in favorable group, p<0.003), including platybasia (1), skull base tumor (1), Chiari I malformation (1), diffuse mild thickening and enhancement of dural and epidural layer of thoracic spine (1), pituitary enlargement (3), sagging brain (3) and subdural hemorrhage (4). In the non-favorable group, 13 out of 23 patients (57%) showed pachymeningeal enhancement in brain MRI (2 patients in favorable group, p<0.001). CONCLUSIONS: Brain MRI abnormalities were more frequently related with non-favorable outcomes in SIH. Pachymeningeal enhancement in particular could suggest an unfortunate prognosis.
Blood Patch, Epidural ; Brain* ; Headache ; Hematoma, Subdural ; Humans ; Intracranial Hypotension* ; Magnetic Resonance Imaging* ; Platybasia ; Prognosis* ; Recurrence ; Retrospective Studies ; Skull Base ; Spine

BACKGROUND: The demographics of the stroke population consist mainly of elderly patients. Transcranial Doppler is an effective method for intracranial flow detection, but can be limited due to the poor temporal window (PTW), which is frequent in the elderly. Therefore, we investigated whether the low frequency 1.6-MHz probe can be useful to improve flow detection for PTW. METHODS: All 201 participants had a history of transient ischemic attack or minor ischemic stroke. Firstly, we analyzed the success rate of recording intracranial blood flow via temporal window by using 2.0-MHz (high frequency, HF) and 1.6-MHz (low frequency, LF) probes for a maximum of 10 minutes. Secondly, mean flow velocity (MFV) and pulsatile index (PI) of insonated spectrum of 25 patients with good temporal window were compared between the two probes. RESULTS: Seventy-eight patients were female and the mean age was 64.1+/-12.4 years. Among 402 windows, 125 (31.1%) were undetected when using the 2.0-MHz probe. Fifty-five patients were detected for the spectrum by the 1.6-MHz probe. However, 70 (17.4%) still remained as PTW. Between the two probes, there were no significant differences of variables from the middle cerebral arteries: MFVs (HF 61.0+/-14.1 vs. LF 61.3+/-14.8 cm/sec, p=0.403 in the right; HF 59.6+/-13.4 vs. LF 59.3+/-13.3 cm/sec, p=0.232 in the left) and PIs (HF 0.82+/-0.17 vs. LF 0.82+/-0.18, p=0.929 in the right; HF 0.82+/-0.20 vs. LF 0.83+/-0.17, p=0.605 in the left). CONCLUSIONS: Conventional 2.0-MHz probe had relatively high proportion of PTW but 1.6-MHz probe was feasible to improve flow detection for PTW.
Aged ; Cerebral Arteries ; Demography ; Female ; Humans ; Ischemic Attack, Transient ; Middle Cerebral Artery ; Stroke ; Temporal Bone ; Ultrasonography, Doppler, Transcranial

Korea health care system and public welfare system have been changed slowly and developed step by step. It makes efforts to promote the welfare of the people, but does not provide good benefits for geriatric patients. In 2008, it was incompletely set up the long term care system in Korea. And the Law on Long-Term Care Insurance for Elderly was officially announced in Nov. 2011 and the law for dementia, Dementia Management Act, was drafted in Feb. 2012. Though there were many laws, the concept of long-term care hospital and geriatric care facility is still obscure. In long-term care hospital, there are big expansion of the number of beds and institutions in Korea. Over a half of the patients in long-term care hospitals are suffered from mixed neurological diseases. Especially, dementia is the comorbid state with other chronic geriatric diseases. In view of the aging population, the role of neurologist will continue to grow. So neurologists need more special training for the needed skills and competencies, and the specific care settings program in long-term care hospital. In this review, the history and current state of long-term care hospital, related laws, and the role of neurologist will be discussed.
Aged ; Aging ; Delivery of Health Care ; Dementia ; Humans ; Insurance, Long-Term Care ; Jurisprudence ; Korea ; Long-Term Care*

Though the polymyositis, one of the common adult-onset myopathy, is treatable, the diverse clinical manifestation and course sometimes lead the clinician to incorrect diagnosis. Here we report a case of polymyositis, presented with atypical features and diagnosed as muscular dystrophy on muscle biopsy previously, but showed marked clinical improvement after combined treatment with prednisolone and methotrexate.
Biopsy* ; Diagnosis ; Methotrexate ; Muscular Diseases ; Muscular Dystrophies* ; Polymyositis* ; Prednisolone