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 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.

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.

[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.

[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 elderly-onset sarcoid myopathy with features resembling polymyositis].

A 75-year-old woman was admitted with a history of acute muscle weakness of her legs for two weeks. Physical examination showed no abnormal findings. Neurological examination revealed symmetrical proximal muscle weakness of legs with muscle pain on grasping. Laboratory data showed normal serum creatine kinase level and marked increases in the levels of serum ACE and soluble interleukin 2. Electromyography showed no myopathic changes. MRI T2 weighted imaging (T2WI) imaging for femoral skeletal muscle demonstrated scattered high and low intensity signals. Muscle biopsy from the right rectus femoris muscle showed granuloma with giant cells of Langhans. She was given a diagnosis of sarcoid myopathy, and motor weakness and abnormal intensity signals on T2WI in this patient were dramatically improved with oral administration of prednisolone (40 mg/day).

[Late-onset leukoencephalopathy without hypertension in a case of young-adult-onset alopecia and spondylosis: a variant of CARASIL?].

We report herein a 61-year-old man with diffuse leukoencephalopathy, subcortical infarcts and cervical and lumbar spondylosis. Medical history included baldness and lumbar spondylosis at young-adult onset. His parents were consanguineous (cousin). He had been experiencing severe lumbago since 20-years-old, with hair loss starting around the same time. He noticed dysarthria and gait disturbance at 59-years-old. He was admitted to our hospital at 61-years-old with aggravation of gait disturbance. On admission, no abnormalities were evident on physical examination except for diffuse baldness. Neurological findings included mild dementia, bilateral hyperreflexia, paraparesis, right Babinski's sign, and pseudobulbar palsy. Blood pressure was normal. T2-weighted imaging of the brain revealed diffuse high-intensity in the periventricular white matter and subcortical infarcts in the brainstem and bilateral basal ganglia. Marked lumbar deformations were observed on spinal MRI. Clinical features in this case met the criteria for cerebral autosomal recessive arteriopathy with subcortical infarctions and leukoencephalopathy (CARASIL), apart from late onset of cerebral infarction.

[A case of subacute necrotizing lymphadenitis with recurrent aseptic meningitis 11 years after the first episode].

We report a 29-year-old man with subacute necrotizing lymphadenitis (SNL) associated with recurrent aseptic meningitis following an 11-year remission period. In both episodes, headache and fever were followed by lymphadenopathy, with increased serum IgE level. Although pleocytosis in cerebrospinal fluid was confirmed at admission in the first episode, it appeared at one week after admission in the second episode. Administration of glucocorticoid was effective for treating meningitis. The present case suggests a pathomechanism for SNL that involves both an immunological background and an acute viral infection as triggers of exacerbation of aseptic meningitis.

[Pure amnesia due to the right capsular genu infarction].

A 77-year-old right-handed woman was admitted to our hospital with memory disturbance. Neurological examination was normal except for amnesia. Neuropsychological tests showed severe impairments in verbal and visual memories. Brain MRI revealed a fresh lacunar infarction in the genu of the right internal capsule. Decreased perfusion in the right thalamus and frontal lobe on (99m)Tc-ECD SPECT was attributable to disconnection of the thalamo-cortical tract by infarction. We consider that the patient's amnesia in this case was induced by infarction of the capsular genu, which includes some fibers from the anterior and inferior thalamic peduncles. Our findings demonstrated that lacunar infarction in the genu of the right internal capsule caused severe and persistent amnesia.

[Autoimmune encephalitis with anti-glutamate receptor antibody presenting as epilepsia partialis continua and action myoclonus: a case report].

A 19-year-old man was admitted to our hospital with tremor and myoclonus that appeared after several episodes of consciousness disturbance and generalized convulsions. While steroid therapy resolved these symptoms, epilepsia partialis continua (EPC) and action myoclonus developed. Clobazam improved the EPC, but action myoclonus persisted. Oral tandospirone (30 mg/day) was given because 5-hydroxyindole acetic acid (5-HIAA) was markedly decreased in the cerebrospinal fluid (CSF). After 10 days of this therapy, most action myoclonus disappeared and he could perform fine motor skills. Although the MR structural images were unremarkable, cerebral SPECT showed decreased uptake in the left thalamus and bilateral frontal lobes. The antibody against glutamate receptor subunit epsilon2 was positive in the CSF. This is the first report of autoimmune encephalitis with anti-glutamate receptor antibody presenting as low level of 5-HIAA in the CSF. Tandospirone was effective for action myoclonus.

[A suspicious case of central neurosarcoidosis, manifesting as a progressive gait disturbance with a lesion in the central gray matter of the midbrain, accompanying an elevated level of angiotensin converting enzyme in the cerebrospinal fluid].

We present a suspicious case of central neurosarcoidosis that presented with progressive gait disturbance probably caused by central vestibular dysfunction. And this case showed elevated level of angiotensin converting enzyme (ACE) in the cerebrospinal fluid, compared with the average level of two cases with acute inflammatory demyelinating neuropathy syndrome and four cases of multiple sclerosis. A 33-year-old man was admitted to our hospital with chief complaint of a gait disturbance that had appeared 3 years prior to the admission. And the symptom had exacerbated in these 3 months. Except for the gait disturbance and positive Romberg's sign, no neurological abnormality was detected. The findings of the cerebrospinal fluid test supported the diagnosis of meningitis. An increased level of angiotensine converting enzyme was detected when compared with our previous samplings from two cases of Guillain Barré syndrome and four cases of multiple sclerosis. With T1 weighted imaging of brain MRI, a high intensity lesion with gadolinium enhancement was identified in the central gray matter of the midbrain. Scan of the chest confirmed bilateral hilar lymphadenopathy. Based on these findings and the patient's clinical course, central neurosarcoidosis was suspected. The patient's symptoms improved dramatically after the administration of corticosteroid. The enhancement of the central gray matter ameliorated, and the ACE level of the CSF was decreased to the level of the demyelinating disorders.

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.

Effect of L-DOPA on nitric oxide production in striatum of freely mobile mice.

In Parkinson's disease, nitric oxide (NO) and other free radicals are thought to be involved in neuronal degeneration. Furthermore, L-DOPA is suggested to have a cytotoxic action on dopaminergic neurons. We studied 24-h NO production and the effect of L-DOPA on this in freely mobile mice using in vivo microdialysis. A microdialysis probe was implanted into the right striatum 12 h before the experiment. This dialysis probe was perfused with Ringer solution for 100 min, then with 20, 50, or 100 nM L-DOPA for 20 min, and finally with Ringer solution. Dialysate fractions were collected every 20 min for 4 h. Production of nitrite and total NO were significantly higher during daytime than during nighttime. Nitrate production was increased significantly by L-DOPA. NO production in the striatum appears to exhibit a diurnal rhythm and to increase with exposure to L-DOPA.

[Sweetness dysgeusia in a case of SIADH caused by lung carcinoma].

A 56-year-old woman, with dysgeusia in which nearly all food was felt as sweet, was admitted to our hospital seeking for treatment. Serum sodium concentration was 113 mmol/L, but serum creatinine, zinc, urea nitrogen, and potassium, as well as blood glucose, were all within normal ranges. Dysgeusia disappeared when serum sodium level was normalized, but recurred when hyponatremia relapsed. She was diagnosed as having large cell lung carcinoma. We considered that the cause of hyponatremia was inappropriate secretion of antidiuretic hormone (SIADH) due to lung carcinoma. Miraculin is one of taste-modifying substances which fits the sweet receptor site and induces a strong sweet taste. We considered that taste-modifying substances same as miraclin are involved in the pathophysiology of this disease.

[A case of acute idiopathic autonomic neuropathy improved by intravenous immunoglobulin].

We describe a case of acute idiopathic autonomic neuropathy (AIAN) in which intravenous administration of immunoglobulin (IVIg) proved effective. A 32-year-old man was admitted with orthostatic dizziness. Fever and headache first developed 24 days earlier, and persisted for 10 days, when orthostatic dizziness developed and prevented him from walking. Hypohidrosis, constipation and impotence also developed. Neurological examinations revealed no abnormalities. Cerebrospinal fluid obtained showed pleocytosis (26/microl) and an increased level of protein (70mg/dl). A head-up tilt test revealed that blood pressure decreased from 120/60mmHg when supine to 60/ 40 mmHg in a head-up position, and the patient complained of dizziness. Plasma noradrenaline concentration was 26pg/ml when supine and 44pg/ml in a head-up position. Results of MIBG cardiac scintigraphy were normal. Dizziness disappeared after initiating IVIg (0.4 g/kg/day). A head-up tilt test was performed 7 days after IVIg, revealing blood pressures of 106/61mmHg when supine and 103/71mmHg in a head-up position. These results suggest that IVIg should be considered as a choice to treat early AIAN.

[A case of isolated hypoglossal nerve palsy probably due to dural arteriovenous fistula].

We report here a case of isolated hypoglossal nerve palsy probably caused by dural arteriovenous fistula (DAVF). A 51-year-old woman was admitted to our hospital complaining headache, tinnitus, and tongue atrophy. Three years before, she first experienced right-sided pulsatile headache and tinnitus which persisted until admission. One week before, she noticed her tongue deviated to right. On admission, physical and neurological examinations showed no abnormal findings except for bruits on right neck and bilateral orbital areas, and atrophy of right tongue. Brain MRI was not remarkable. MRA and conventional angiography disclosed DAVF. We conclude that differential diagnoses for isolated hypoglossal paralysis should include DAVF.

[Reversible posterior leukoencephalopathy syndrome in Hashimoto's encephalopathy: a case report].

A 45-year-old woman with breast cancer was admitted to our hospital because of several episodes of disturbed consciousness and generalized convulsions. While these symptoms resolved quickly, dysphagia and bilateral blepharoptosis persisted. Neurological findings were improved by steroid therapy. MRI on the first hospital day showed T2/FLAIR high intensity lesions in both occipital lobes, but these lesions diminished on the 8th day, indicating reversible posterior leukoencephalopathy syndrome. A new lesion appeared in the left temporal lobe on the 8th day. A diagnosis of Hashimoto's encephalopathy (HE) was made due to the following features: 1) encephalitis not due to herpes simplex virus, 2) high titers of antithyroid antibodies in serum, 3) marked effectiveness of steroid therapy, and 4) antibodies against the amino terminal of alpha-enolase, a specific antigen for HE.

[Case of straight sinus venous thrombosis presenting as depression and disorientation due to bilateral thalamic lesions].

A 45-year-old man was admitted to our hospital because of progressive inactivity and mild disturbance of consciousness which appeared two weeks ago. Brain CT revealed symmetric hypointensity of bilateral thalamus, and the lesion appeared hyperintensity on T2 weighted MRI image. He was first considered as immune-mediated cerebritis, and steroid pulse therapy was applied, but the clinical features were not improved. The diagnosis of cerebral venous thrombosis was established, when MR venography (MRV) showed severe stenosis in straight sinus. Consciousness was improved after the start of anticoagulation therapy, but mild dementia was remained as a sequela. MRV was useful to distinguish straight sinus thrombosis from cerebritis in this case.