Makoto Shiraishi

Progression of Intracranial Major Artery Stenosis is Associated with Baseline Carotid and Intracranial Atherosclerosis

AIM Intracranial atherosclerotic major artery stenosis (IMAS) is associated with a high risk of ischemic stroke. Carotid ultrasound (US) has been widely used to evaluate an individuals atherosclerotic burden, but no information is available on whether the carotid US findings are associated with IMAS progression. The aim of the present study was to identify the associations among traditional risk factors, the duplex carotid US findings and IMAS progression in patients with varying degrees of carotid atherosclerosis. METHODS All patients who underwent a set of imaging studies (MRI, MRA and carotid US) in our outpatient clinic were screened. A total of 101 patients with a mean age of 75.0±10.6 years, who received the same imaging studies with a mean interval of two years, were studied. In each patient, the extent of stenosis of three arteries (both middle cerebral arteries [MCAs] and the basilar artery [BA]) was classified into five grades. The total score of the three arteries was calculated as the global stenosis score (GSS). The progression of IMAS was defined as worsening of stenosis by ≥1 grade on final MRA. The maximum IMT (maxIMT), plaque findings and carotid stenosis were measured by carotid US. A multivariate stepwise logistic regression analysis was used to identify independent predictors of IMAS progression. RESULTS Follow-up MRA revealed IMAS progression in 12 patients (11.9%). The logistic regression analysis demonstrated that the baseline GSS (p=0.008) and carotid stenosis ≥70% on the baseline carotid US (p=0.023) were significantly associated with IMAS progression. CONCLUSIONS The baseline severity of intracranial and extracranial atherosclerosis was significantly associated with the progression of IMAS.

Steroid-resistant Tolosa-Hunt syndrome with a de novo intracavernous aneurysm: A case report

Background: We report a case of steroid-resistant Tolosa–Hunt syndrome (THS) with recurrent bilateral painful ophthalmoplegia, accompanied with sphenoid sinusitis, pituitary abscess, and an aneurysm arising from the cavernous portion of the internal carotid artery. Case Description: A 53-year-old woman suffered severe left painful ophthalmoplegia. A magnetic resonance image (MRI) revealed thickness of the left cavernous sinus (CS). Steroid was administrated under the diagnosis of THS, and the symptom transiently diminished. However, painful ophthalmoplegia fluctuated bilaterally after tapering the steroid. An MRI showed development of bilateral cavernous lesions associated with sphenoid sinusitis, pituitary abscess, and an aneurysm in the left C4 segment. Biopsy and drainage of the lesions were performed with an endoscopic transsphenoidal procedure. The histological examination showed nonspecific granulomatous inflammation. The methotrexate (MTX) was effective to reduce the CS and pituitary lesions; however, the aneurysm slightly increased and remained unchanged in size thereafter. Conclusions: To our knowledge, this is the first report of a growing de novo C4 aneurysm in THS. Surgical intervention and administration of MTX should be attempted in steroid-resistant THS. Careful observation with serial MRI and MR angiography is important to manage the complicated THS.

Usefulness of switching to cabergoline from other dopamine agonists in patients with advanced Parkinson’s disease

Summary. Problems associated with long-term treatment of advanced Parkinson’s disease (PD) include motor complications and psychotic and autonomic symptoms. We switched patients from bromocriptine (BR) or pergolide (PER) to cabergoline (CB) therapy and investigated CB’s usefulness in alleviating such problems. Subjects were 30 patients (mean age 68.2 years; 13 receiving BR, 17 PER) with PD complicated by effects of long-term treatment but in whom their dose of dopamine (DA) agonist was contraindicated due to adverse reactions. Patients were switched to CB over a 2–4-week period. Hoehn-Yahr and Unified Parkinson Disease Rating Scale (UPDRS) I–IV “on” and “off” scores improved in both the BR and PER groups. CB was not discontinued due to adverse reactions in any patient. In conclusion, switching to CB is useful in patients in whom it is problematic to increase their dose of DA agonist due to motor complications or psychotic symptoms of advanced PD.

Forward flexion of trunk in Parkinson's disease patients is affected by subjective vertical position

Purpose No method has been established to evaluate the dissociation between subjective and objective vertical positions with respect to the self-awareness of postural deformity in patients with Parkinson’s disease (PD). The purpose of this study was to demonstrate, from the relationship between an assessment of the dissociation of subjective and objective vertical positions of PD patients and an assessment based on established PD clinical evaluation scales, that the dissociation regarding vertical position is a factor in the severity of the forward flexion of trunk (FFT). Methods Subjects were 39 PD patients and 15 age-matched healthy individuals (control group). Posture was evaluated with measurement of FFT angle during static standing and the subjective vertical position (SV) of the patient. For evaluation of motor function, the Modified Hoehn & Yahr scale, Unified Parkinson’s Disease Rating Scale (UPDRS), 3-m Timed Up and Go Test (TUG), and Functional Reach Test (FRT) were used. Results In PD patients, FFT angle in the 3rd tertile of patients was 13.8±9.7°, significantly greater than those in the control group and the 1st and 2nd tertiles of PD patients (control group vs 3rd tertile, p = 0.008; 1st tertile vs 3rd tertile, p<0.001; 2nd vs 3rd tertile, p = 0.008). In multiple regression analysis for factors in the FFT angle, significant factors were SV, disease duration, and the standard deviation of each SV angle measurement. Conclusion The dissociation between SV and objective vertical position affects the FFT of PD patients, suggesting an involvement of non-basal ganglia pathologies.

Overnight Monitoring of Turnover Movements in Parkinsons DiseaseUsing A Wearable Three-Axis Accelerometer

Background: In patients with Parkinsons disease (PD), the impairment of voluntary and involuntary movement during sleep might affect their natural sleep and quality of life; however, there is no reliable method to evaluate turnover movements during sleep. We aimed to clarify whether overnight monitoring of turnover movements in bed using a wearable three-axis accelerometer is a feasible and reliable tool for evaluating the impact of motor complications during sleep in PD. Methods: The number of turnover movements in bed was counted based on the graphic pattern in the X, Y, and Z axis using threshold values in each axis to discriminate turnover movements from other movements mainly associated with respiration or cough. These threshold values were defined by the recordings of various turnover movements in normal volunteers. Overnight monitoring of turnover movements in bed from 9:00 pm to 7:00 am was performed in 7 normal volunteers and 5 patients (mean age, 76.4 ± 4.6 yrs, Hoehn-Yahr stage, 3.6 ± 0.5; duration of disease, 8.8 ± 5.6 yrs). In patients with PD, monitoring was performed before and after adjustment of anti-Parkinson medications. Results: The number of turnover movements was significantly more restricted in PD before drug control than in control subjects (p=0.005). The median number of overnight turnover movements increased from 0 to 5 times after drug control in patients with PD. The number of overnight turnover movements increased significantly in all 5 patients after adjusting their anti-Parkinson medications (p=0.041). Conclusion: Overnight monitoring of turnover movements in bed using a wearable three-axis accelerometer is feasible. Further studies are warranted to evaluate the impact of movement disorders during sleep on patients with PD.

Mononeuritis multiplex in a patient with cutaneous arteritis diagnosed by skin biopsy

A 55-year-old man was admitted with paralysis of the left lower leg. He had purpura in the left lower extremity for three years, left calf pain for two years, and dysesthesia in the left plantar region and first toe for one year. A physical examination revealed livedo reticularis on the left leg and mononeuritis multiplex was diagnosed in the bilateral tibial and left peroneal nerve area. Anti-neutrophil cytoplasmic antibody was negative. A nerve conduction study showed decreased amplitude of compound muscle-action potential in the bilateral tibial and the left peroneal nerve, sensory nerve action potential in the bilateral sural nerve. A skin biopsy revealed inflammatory cells on blood vessel walls and cutaneous arteritis was diagnosed. Cyclophosphamide pulse therapy with steroid and anti-coagulation improved the neurological symptoms. A skin biopsy should be considered when patients present with mononeuritis multiplex in the lower extremities and cutaneous findings such as livedo reticularis in the symptomatic area.

Impact of inability to turn in bed assessed by a wearable three-axis accelerometer on patients with Parkinson's disease

Background Difficulty turning over in bed is a common night-time symptom in Parkinson’s disease (PD). We aimed to quantitatively evaluate overnight turnover movements using a three-axis accelerometer and to investigate whether inability to turn in bed is related to daytime sleepiness, sleep quality, and depressive mood in PD patients. Methods We examined 64 patients with PD (mean age, 73.3±8.21 years; modified Hoehn-Yahr [mH-Y] stage, 3.0±1.0; disease duration, 7.2±6.3 years; unified Parkinsons disease rating scale [UPDRS], 36.9±18.3). Overnight monitoring of turnover movements using a wearable three-axis accelerometer was performed in all patients. Nocturnal kinetic parameters including total time recumbent, total time supine, number of turnover movements, and mean interval between turnover movements were obtained. Daytime immobility was assessed using the Barthel index (B-I), UPDRS, and mH-Y stage. Patients were also assessed with the Epworth Sleepiness Scale (ESS), Parkinson’s Disease Sleep Scale-2 (PDSS-2), and Beck Depression Inventory (BDI). Results Number of turnover movements in bed correlated negatively with disease duration (r = -0.305; p<0.05), L-dopa-equivalent dose (r = -0.281; p<0.05), mH-Y staging (r = -0.336; p<0.01), total score of UPDRS (r = -0.386; p<0.01) and positively with B-I score (r = 0.365; p<0.01). Number of turnover movements in bed was generally inconsistent with awareness of turnover movement impairment as evaluated by PDSS-2 Item 9 scores, but patients who were never aware of impaired turnover movements showed ≥5 turnover movements overnight. Multivariate logistic regression analyses revealed no correlations between number of nocturnal turnover movements in bed and BDI, ESS, or PDSS-2. Use of anti-psychotic drugs was associated with ESS (p = 0.045). UPDRS was associated with PDSS-2 (p = 0.016). Conclusion Decreased number of turnover movements may not be a direct determinant of daytime sleepiness, sleep disorders, or depressive mood in PD patients. Use of anti-psychotic drugs and higher UPDRS score are factors significantly associated with daytime sleepiness and uncomfortable sleep, respectively.

Nerve conduction study of lower extremities in cutaneous arteritis patients with neurological manifestations

Some patients originally diagnosed with cutaneous arteritis (CA) could develop additional disease manifestations, including peripheral neurological involvement. We evaluated the biological neurological parameters among CA patients who underwent nerve conduction studies for neurological involvement in the lower extremities. We reviewed 164 patients who were originally diagnosed with CA at our dermatology department between 2004 and 2015. Seventeen (10.4%) of the CA patients underwent further nerve conduction studies to determine their peripheral neurological manifestations, primarily in the lower extremities, in our neurology division. The frequency of low compound muscle action potential (CMAP) was significantly higher compared with that of delayed latency in both the peroneal nerve and sural nerve based on nerve conduction studies. The frequency of low CMAP was significantly higher compared with that of prolonged distal latency in both the peroneal and sural nerves. We suggest that impairment of the nerve axon pathways in the peroneal and sural nerves could result in the peripheral neurological manifestations in the lower extremities in CA patients.

Reversible Cerebral Vasoconstriction Syndrome Presenting with Transient Global Amnesia

A 65-year-old man who had been diagnosed with transient global amnesia (TGA) 15 years previously was admitted to hospital with complaints of amnesia and headache. His symptoms improved on day-2. The initial brain MRI and electroencephalography findings were normal. He was diagnosed with a recurrence of TGA and discharged. However, he returned with right leg weakness and complained of a thunderclap headache. MRI demonstrated subarachnoid hemorrhage and multifocal segmental narrowing of the left posterior cerebral artery (PCA) and large intracranial arteries, and he was diagnosed with reversible cerebral vasoconstriction syndrome (RCVS). He was discharged on day-30 without any neurological deficits. This case suggested that TGA should be interpreted as one of the symptoms of RCVS or a prodromal symptom of RCVS.

A case of refractory IgG4-related peripheral neuropathy with severe axonal damage.

A 78-year-old man presented complaining of tingling and pain. Neurological examination revealed dysesthesia and hypothermesthesia below both knees and areflexia in the lower extremities. Laboratory data revealed elevated serum levels of immunoglobulin IgG4 and para-aortic, and mesenteric lymphadenopathy was evident on plain computed tomography of the abdomen. Microscopic findings of a bone marrow biopsy specimen showed occlusion of blood vessels with IgG4-positive plasma cells. IgG4-related disease was diagnosed because the bone marrow biopsy exhibited > 10 IgG4-positive plasma cells per high-power field. Treatment was initiated with prednisolone starting at 30 mg/day, but no improvement in neurological symptoms was achieved. Sural nerve biopsy demonstrated obstructive thromboangiitis with severe loss of myelin and axons. Further investigations are needed to elucidate the relationship between obstructive thromboangiitis and steroid-resistant IgG4-related peripheral neuropathy.