Polycystic subdural hygroma associated with immunoglobulin G4-related intracranial hypertrophic pachymeningitis: a case report.

BACKGROUND: Recent studies have examined hypertrophic pachymeningitis as an IgG4-RD. However, there are no reports of immunoglobulin G4 (IgG4)-related hypertrophic pachymeningitis with polycystic subdural hygroma. CASE PRESENTATION: A 56-year-old man presented to the hospital with complaints of a persistent, pulsatile, occipital headache and general malaise. Magnetic resonance imaging of the brain revealed hypertrophic pachymeningitis with polycystic subdural hygroma and hematoma. Based on the dural biopsy findings and exclusion of other diseases, the patient was diagnosed with immunoglobulin G4 (IgG4)-related hypertrophic pachymeningitis. IgG4-related diseases may cause subdural hygroma more commonly than other diseases that cause hypertrophic pachymeningitis. CONCLUSIONS: This is the first case report discussing polycystic subdural hygroma and hematoma with IgG4-related hypertrophic pachymeningitis.

Isolated Hypoglossal Paralysis Caused by Ischemic Infarction in the Centrum Semiovale.

In the present report, we discuss the case of a 66-year-old woman with isolated unilateral hypoglossal paralysis due to cerebral infarction in the centrum semiovale. To date, it has hardly been discussed where the corticolingual tract passes through in the centrum semiovale. Brain magnetic resonance imaging revealed a small ischemic infarction in the contralateral centrum semiovale. We could demonstrate a route of the corticolingual tract.

A case of urinary retention in the early stages of herpes simplex virus type-1 encephalitis.

A 70-year-old man developed urinary retention in the early stages of herpes simplex virus (HSV) type-1 encephalitis. A nerve conduction study suggested latent myeloradiculitis. This is the first report of human herpes simplex virus-1 encephalitis followed by urinary retention at early stage from the onset like the Elsberg syndrome. Although relatively few similar cases have been reported, we consider that urinary retention is common in HSV-1 encephalitis, in which disturbances of consciousness usually require bladder catheterization from the onset. We further emphasize that urinary retention may occasionally occur in early stages of HSV-1 encephalitis, with a significant possibility of recovery.

A case of idiopathic pure sudomotor failure associated with prolonged high levels of serum carcinoembryonic antigen.

We report a case of idiopathic pure sudomotor failure (IPSF) in which serum carcinoembryonic antigen (CEA) levels elevated at onset, and remained high while anhidrosis lasted. We considered that changes in serum levels of CEA were related to the disease activity of IPSF.

[Neuroanatomy of Isolated Body Lateropulsion].

Axial body lateropulsion, a phenomenon where the body is pulled toward the side of the lesion, with tendency of falling down, is the well-known transient feature of lateral medullary syndrome. In some cases, axial body lateropulsion occurs without vestibular and cerebellar symptoms (isolated body lateropulsion:[iBL]). Patients with iBL have a lesion located in the spinocerebellar tract, descending lateral vestibulospinal tract, vestibulo-thalamic pathway, dentatorubrothalamic pathway, or thalamocortical fascicle. This review deals with the anatomic basis and clinical significance of iBL.

Evaluation of the Differences in the Effects of Antihypertensive Drugs on Blood Pressure Variability by 24-Hour Ambulatory Blood Pressure Monitoring in Chronic Cerebrovascular Disease.

BACKGROUND: It has been suggested that antihypertensive drug therapy is attributable to the lower blood pressure variability, we investigated the effects of 4 classes of antihypertensives on the blood pressure variability; in addition, we also compared the effects among 4 calcium channel blockers. METHODS: We measured the 24-hour blood pressure variability in 309 patients with a history of cerebrovascular disease treated with angiotensin-converting enzyme inhibitor, angiotensin receptor blocker, ß blocker, or calcium channel blocker. RESULTS: The daytime blood pressure variability treated with ß blockers (14.3 ± 3.1) was higher than that treated with an angiotensin receptor blockers (11.5 ± 3.1) or calcium channel blockers (12.6 ± 3.4) in patients with cerebrovascular disease (P < .05). In the analysis of the patient distribution of blood pressure variability, patients receiving ß blockers occurred more frequently in the higher blood pressure variability (P = .0023). Treatment with angiotensin receptor blockers and cilnidipine, which blocks N-type calcium channels, was shown to be more frequently associated with the lower blood pressure variability (P = .0202 and .0467). The mean blood pressure of patients grouped by distribution of blood pressure variability was found to be independent to blood pressure variability, for any of the antihypertensive drugs or calcium channel blockers examined. CONCLUSIONS: From the results, it is suggested that angiotensin receptor blocker and calcium channel blockers rather than ß blockers may be more favorable for blood pressure management in patients with cerebrovascular disease. Among the calcium channel blockers, cilnidipine may be more favorable than other calcium channel blockers.

A case of α-synuclein gene duplication presenting with head-shaking movements.

BACKGROUND: PARK4 is a candidate locus for familial Parkinson's disease (PD), combined with multiplication of the α-synuclein gene (SNCA). The eventual phenotype is dependent on the copy number of SNCA. Mutations in leucine-rich repeat kinase 2 (LRRK2) are also causative of parkinsonism. This report describes a man who presented at our hospital complaining of a stagger after running and difficulty in handling the mouse of a personal computer, having suffered tremors since his twenties. Nine months after treatment and discharge, he developed titubation and began to drag his right foot. METHODS: We examined the patient's family pedigree for SNCA dosage, using quantitative polymerase chain reaction. We also screened this pedigree for mutations in parkin and LRRK2, using gene-sequencing techniques. RESULTS: We identified the proband, his sister, and his paternal uncle as carrying a duplication of SNCA. In addition, we found that the proband and his mother carried the G2385R variant of the LRRK2, a strong risk factor for PD in Asians and the rare V1450I variant, although only the proband showed symptoms of parkinsonism. No mutations were found in parkin. CONCLUSIONS: The combination of SNCA gene duplication and LRRK2 G2385R variant may explain the early onset of disease in this patient.

[Autonomic features in Parkinson disease].

Nonmotor symptoms such as autonomic and neuropsychiatric dysfunctions, are commonly seen in Parkinson disease (PD). Recent studies have shown that PD is accompanied by cardiac sympathetic denervation and constipation even in the early stage. Neuropathological studies confirmed changes in the cardiac sympathetic nerves and the gastrointestinal tract. These findings suggest that PD neuropathology may occur first in the peripheral autonomic pathways and extend to the central autonomic pathways, in agreement with the "Braak theory". This article will reviews the symptoms and pathophysiology of gastrointestinal dysfunction, urinary disturbance, sexual dysfunction, sweating dysfunction, pupillary autonomic dysfunction, and orthostatic and postprandial hypotension in PD patients, and discuss to organ selectiveness in autonomic dysfunction in PD.

Oculopharyngeal muscular dystrophy associated with dementia.

We report genetically confirmed heterozygote oculopharyngeal muscular dystrophy (OPMD) accompanied by dementia, suggesting a possible causal association between OPMD and dementia. The proband first noticed bilateral ptosis, dysphagia, and proximal dominant muscle weakness in the lower extremities at age 53. Ten years later, she was found to have dementia with a score of 10/30 on the mini-mental state examination (MMSE). On PABPN1 gene analysis, the GCN repeat was expanded 17 times in one allele. In addition, the proband's younger brother exhibited myopathy and dementia. To our knowledge, this is the first report of genetically confirmed heterozygote OPMD associated with dementia.

[A case of occipital epilepsy with anti-GluRepsilon2 antibody in cerebrospinal fluid, presenting as repeated visual disturbance and headache].

A 78-year-old man was admitted to our hospital with repeated attacks of headache and visual hallucinations, which had begun 10 days before. He also displayed left hemispatial neglect and left homonymous hemianopsia during attacks. Brain magnetic resonance imaging (MRI) showed an abnormal high-intense area in the right occipital lobe on diffusion weighted imaging (DWI) and fluid attenuated inversion recovery (FLAIR) weighted imaging; this lesion was demonstrated as an area of hyperperfusion on ECD-single photon emission computed tomography (SPECT) and hypoperfusion on 123I-BZ-SPECT. Electroencephalography during an attack demonstrated epileptogenic discharges in the right occipital region. Acute urinary retention due to meningoencephalitis appeared 2 weeks after onset of the first attack. Autoantibodies against glutamate receptor epsilon2 were detected in cerebrospinal fluid. We diagnosed the patient with occipital epilepsy due to anti-NMDA receptor antibody encephalitis. Epileptic attacks diminished and MRI and SPECT findings improved following two administrations of intravenous bolus steroid therapy.

[A case of neuro-neutrophilic disease presenting with 5 months' with cognitive decline, meningoencephalitis, and marked systemic inflammatory findings, and diagnosed with brain biopsy].

A 63-year-old man was admitted to our hospital with cognitive decline. On admission, he had a fever and mild cognitive dysfunction, suggesting chronic meningoencephalitis. Apart from a mild increase in serum C-reactive protein level and marked neutrophilia, laboratory findings were unremarkable. Brain magnetic resonance (MR) imaging showed multiple small T2-hyperintense lesions in the white matter. Systemic evaluations for infectious organisms, autoantibodies, and malignancy were all negative. For 5 months we conducted therapeutic trials of various antibacterial, antifungal, and antituberculous drugs, but these were completely ineffective, and both meningoencephalitis and inflammatory signs persisted. Repeated brain MRI during the clinical course showed growth of the white matter lesions and progressive cerebral atrophy. C11-methionine positron emission tomography demonstrated a bright focus in the right frontal lobe, and this was biopsied. Key neuropathological findings were neutrophilic infiltration in the subarachnoid space and the frontal lobe without necrotic angiitis. These findings confirmed the diagnosis of neuro-neutrophilic disease, although skin tissue findings characteristic of Sweet disease and a B51, B54, or Cw1 HLA-profile were absent. After intravenous bolus administration of steroid and prolonged oral steroid therapy, fever and inflammatory signs diminished and cognitive symptoms improved.

Comparison of the anti-hypertensive effects of the L/N-type calcium channel antagonist cilnidipine, and the L-type calcium channel antagonist amlodipine in hypertensive patients with cerebrovascular disease.

OBJECTIVES: It is known that the risk of cerebral stroke recurrence in post-stroke patients is comparatively higher than in normal subjects, and it is suggested that autonomic nervous system dysfunctions elevate this risk. We investigated the anti-hypertensive effects of cilnidipine, a Ca antagonist which suppresses sympathetic nerve activation, in hypertensives with chronic-stage cerebrovascular disease in a comparison with amlodipine. METHODS: Amlodipine 5-7.5 mg/day, or cilnidipine 5-10 mg/day was administered to 78 hypertensive subjects (greater than 140 mmHg systolic, or 90 mmHg diastolic) undergoing outpatient treatment. Amlodipine or cilnidipine was also administered similarly, to 30 subjects having hypertension associated with a cerebral infarct which occurred more than one month earlier due to cerebral thrombosis or embolism. After 3 months administration, the subjects' blood pressures and pulse rates were recorded with an ambulatory blood pressure monitor over 24 hours. RESULTS: No difference was recognized in patient age, gender, and systolic and diastolic blood pressure before treatment between the groups. In the cilnidipine groups, no difference in average 24-hour or waking systolic blood pressure values was seen between cerebrovascular disease (CVD) subjects and non-CVD subjects, although in the amlodipine groups, CVD subjects had significantly higher blood pressure values than non-CVD subjects. In the cilnidipine group, the coefficient of variation values of pulse rate were significantly higher in CVD subjects than in non-CVD subjects (p<0.05). CONCLUSION: In patients with recent stroke, a Ca antagonist with no sympathetic nerve suppression had weaker blood pressure-lowering effects. Significantly increased pulse rate variability, shown in the CVD subjects administered cilnidipine, suggests that cilnidipine enhanced the parasympathetic function in hypertensive patients with CVD.