The utility of the combined use of 123I-FP-CIT SPECT and neuromelanin MRI in differentiating Parkinson's disease from other parkinsonian syndromes.

BACKGROUND: Neuromelanin magnetic resonance imaging (NmMRI) and 123I-FP-CIT dopamine transporter single photon emission computed tomography (DAT-SPECT) provide specific information that distinguishes Parkinson's disease (PD) from non-degenerative parkinsonian syndrome (NDPS). PURPOSE: To determine whether a multiparametric scoring system (MSS) could improve accuracy compared to each parameter of DAT-SPECT and NmMRI in differentiating PD from NDPS. MATERIAL AND METHODS: A total of 49 patients, including 14 with NDPS, 30 with PD, and five with atypical parkinsonian disorder (APD) underwent both NmMRI and DAT-SPECT and were evaluated. The average (Ave) and the asymmetry index (AI) were calculated in the substantia nigra compacta area (SNc-area), SNc midbrain-tegmentum contrast ratio (SNc-CR), and specific binding ratio (SBR). Cut-off values were determined, using receiver operating characteristic (ROC) analysis, for the differentiation of PD from NDPS on the statistically significant parameters. All cases were scored as either 1 (PD) or 0 (NDPS) for each parameter according to its threshold. These individual scores were totaled for each case, yielding a combined score for each case to obtain a cut-off value for the MSS. RESULTS: The Ave-SNc-area, Ave-SNc-CR, and Ave-SBR in PD were significantly lower than those in NDPS. The AI-SNc-area and AI-SBR in PD were significantly higher than those in NDPS. Of the five parameters, the highest accuracy was 93% for the Ave-SNc-area. For the MSS, a cut-off value of 3 was the accuracy of 96%. Besides, no significant difference was observed between PD and APD on all parameters. CONCLUSION: An MSS has comparable or better accuracy compared to each parameter of DAT-SPECT and NmMRI in distinguishing PD from NDPS.

The Utility of the Combined Use of 123I-FP-CIT and 123I-MIBG Myocardial Scintigraphy in Differentiating Parkinson's Disease from Other Parkinsonian Syndromes.

BACKGROUND: 123I-metaiodobenzylguanidine (MIBG) myocardial scintigraphy and 123I-FP-CIT dopamine transporter single photon emission computed tomography (DAT-SPECT) provide specific information that distinguish Parkinson's disease (PD) from parkinsonian syndromes other than PD (non-PD), including atypical parkinsonian disorder (APD) and non-PD other than APD (nPD-nAPD). The purpose of this study was to determine whether combining DAT-SPECT and MIBG myocardial scintigraphy using multiparametric scoring system (MSS) could improve diagnostic test accuracy in discriminating PD from APD or discriminating PD from nPD-nAPD. METHODS: A total of 52 patients, including 36 PD, eight APD and eight nPD-nAPD, underwent both MIBG myocardial scintigraphy and DAT-SPECT, were evaluated. The heart-to-mediastinum (H/M) ratios (early and delayed), washout-rate (WR), the average (Ave) and asymmetry index (AI) of specific binding ratio (SBR) were calculated. Cutoff values were determined, using ROC analysis, for discriminating PD from APD and for discriminating PD from nPD-nAPD, on five parameters. All cases were scored as either 1 (PD) or 0 (nPD-nAPD or APD) for each parameter according to its threshold in each discrimination. These individual scores were summed for each case, yielding a combined score to obtain a cutoff value for the MSS in each discrimination. RESULTS: For discriminating PD from nPD-nAPD, the highest accuracy was 80% at a cutoff value of 19% for the WR and a cut off value of 2 improved diagnostic accuracy to 84% for MSS. For discriminating PD from APD, the highest accuracy was 86% at a cutoff value of 2.8 for the H/M ratio (late) and a cut off value of 2 showed diagnostic accuracy of 86% for MSS. CONCLUSION: A MSS has comparable or better accuracy compared to each parameter of MIBG myocardial scintigraphy and DAT-SPECT in distinguishing PD from nPD-nAPD or distinguishing PD from APD.

Association between Serum Uric Acid Level and Activity of Daily Living in Japanese Patients with Ischemic Stroke.

BACKGROUND: An association between serum uric acid and outcomes of ischemic stroke has been reported, but the results are controversial. The aim of this study is to clarify how uric acid may affect activities of daily living after acute ischemic stroke. METHODS: Consecutive Japanese patients with acute ischemic stroke were analyzed. Serum uric acid quartiles and activities of daily living at hospitalization and discharge in men and women were examined. Activities of daily living were evaluated using the modified Rankin scale score, and a score of 3 or higher was defined as poor activities of daily living. P values less than .05 were considered significant. RESULTS: A total of 987 patients with acute ischemic stroke (591 men; mean age, 72.3 years) were analyzed in this study. We observed a U-shaped relationship between serum uric acid and poor activities of daily living in both men and women at hospitalization and discharge. Multivariate analysis demonstrated that the first quartile group of serum uric acid was significantly associated with poor activities of daily living in both men and women, using the third quartile group as the reference. CONCLUSIONS: Lower serum uric acid can be a marker for predicting poor activities of daily living in patients with acute ischemic stroke, irrespective of sex.

Intrathecal Isoniazid for Refractory Tuberculous Meningitis with Cerebral Infarction.

A 30-year-old Vietnamese woman, about 19 weeks pregnant, was admitted for acute cerebral infarction with stenosis of the left middle cerebral artery (LMCA), tuberculous meningitis, and miliary tuberculosis. Treatment with heparin, quadruple anti-tuberculosis therapy, and dexamethasone afforded prompt symptomatic improvement. However, she delivered a stillbirth, after which there was recurrence of acute cerebral infarction with LMCA occlusion, sinus thrombosis, and cranial base inflammation. A thrice-weekly 100 mg dose of intrathecal isoniazid (INH) improved the signs of meningeal inflammation. The patient was discharged ambulatory after 7 months. In refractory tuberculous meningitis, multimodal therapy with intrathecal INH and steroids should be considered.

Association between body mass index and outcome in Japanese ischemic stroke patients.

AIM: An association between body mass index (BMI) and stroke outcome have been reported, but the results are controversial. The aim of the present study was to evaluate whether BMI is associated with ischemic stroke outcome. METHODS: Consecutive Japanese acute ischemic stroke patients were analyzed. BMI was categorized as underweight (BMI <18.5 kg/m2 ), normal weight (18.5-24.9 kg/m2 ) and obese (≥25 kg/m2 ). BMI and short-term and long-term outcomes were examined. Short-term outcomes were evaluated using the modified Rankin scale score at hospitalization and discharge; modified Rankin scale ≥3 was defined as a poor outcome. Long-term outcomes were evaluated by all-cause mortality. The recurrence rate was also evaluated in each BMI group. Values of P < 0.05 were considered significant. RESULTS: A total of 1206 acute ischemic stroke patients (760 men; mean age 72.5 years) were analyzed in the present study. There were 111 underweight cases (9.2%), 785 normal weight cases (65.1%) and 310 obese cases (25.7%). The underweight group had a significantly higher rate of poor short and long-term outcomes than the normal weight group. The outcomes of the obese group were not significantly different from those of the normal weight group. Recurrence was not significantly different among the groups. CONCLUSIONS: Lower BMI might be a predictor of poorer short-term and long-term stroke outcomes. Geriatr Gerontol Int 2017; 17: 369-374.

Clinicopathological features of adult-onset neuronal intranuclear inclusion disease.

Neuronal intranuclear inclusion disease (NIID) is a slowly progressive neurodegenerative disease characterized by eosinophilic hyaline intranuclear inclusions in the central and peripheral nervous system, and also in the visceral organs. NIID has been considered to be a heterogeneous disease because of the highly variable clinical manifestations, and ante-mortem diagnosis has been difficult. However, since we reported the usefulness of skin biopsy for the diagnosis of NIID, the number of NIID diagnoses has increased, in particular adult-onset NIID. In this study, we studied 57 cases of adult-onset NIID and described their clinical and pathological features. We analysed both NIID cases diagnosed by post-mortem dissection and by ante-mortem skin biopsy based on the presence of characteristic eosinophilic, hyaline and ubiquitin-positive intanuclear inclusion: 38 sporadic cases and 19 familial cases, from six families. In the sporadic NIID cases with onset age from 51 to 76, dementia was the most prominent initial symptom (94.7%) as designated 'dementia dominant group', followed by miosis, ataxia and unconsciousness. Muscle weakness and sensory disturbance were also observed. It was observed that, in familial NIID cases with onset age less than 40 years, muscle weakness was seen most frequently (100%), as designated 'limb weakness group', followed by sensory disturbance, miosis, bladder dysfunction, and dementia. In familial cases with more than 40 years of onset age, dementia was most prominent (100%). Elevated cerebrospinal fluid protein and abnormal nerve conduction were frequently observed in both sporadic and familial NIID cases. Head magnetic resonance imaging showed high intensity signal in corticomedullary junction in diffusion-weighted image in both sporadic and familial NIID cases, a strong clue to the diagnosis. All of the dementia dominant cases presented with this type of leukoencephalopathy on head magnetic resonance imaging. Both sporadic and familial NIID cases presented with a decline in Mini-Mental State Examination and Frontal Assessment Battery scores. Based on these clinicopathological features, we proposed a diagnosis flow chart of adult-onset NIID. Our study suggested that the prevalence rate of adult-onset NIID may be higher than previously thought, and that NIID may be underdiagnosed. We should take NIID into account for differential diagnosis of leukoencephalopathy and neuropathy.

Atypical Arteritis in Internal Carotid Arteries: A Novel Concept of Isolated Internal Carotid Arteritis.

We presented a 38-year-old woman suffering from acute cerebral infarction due to arteritis limited to bilateral internal carotid arteries without a condition of giant cell arteritis or granulomatosis with polyangitis. Our case is unprecedented and characterized by a young woman with wall enhancement in the internal carotid arteries on contrast-enhanced magnetic resonance imaging (MRI), therapeutic effects of steroids, and positive status for human leucocyte antigen-B39, -B51 and -DR4. These disease characteristics were not in accordance with existing diagnostic criteria of vasculitis, such as Takayasu's arteritis, giant cell arteritis, granulomatosis with polyangiitis, and Behcet's disease. We suggested consideration of a novel "isolated internal carotid arteritis" disease concept.

Acute Motor-dominant Polyneuropathy as Guillain-Barré Syndrome and Multiple Mononeuropathies in a Patient with Sjögren's Syndrome.

A patient with xerostomia and xerophthalmia due to Sjögren's syndrome presented with acute motor-dominant polyneuropathy and multiple mononeuropathy with antiganglioside antibodies. Nerve conduction studies and a sural nerve biopsy revealed the neuropathy as a mixture of segmental demyelination and axonal degeneration. Positive results were obtained for several antiganglioside antibodies. Corticosteroid treatment proved effective. The neuropathy was considered to represent a mixture of polyneuropathy as Guillain-Barré syndrome and multiple mononeuropathy via Sjögren's syndrome. We speculate that Guillain-Barré syndrome occurred in the patient and Guillain-Barré syndrome itself activated multiple mononeuropathy via Sjögren's syndrome.

Three cases of neuromyelitis optica spectrum disorder.

Neuromyelitis optica (NMO) is characterized by attacks of optic neuritis and longitudinally extensive transverse myelitis. Cases positive for aquaporin 4 antibodies are classified to NMO spectrum disorder (NMOSD) which includes cases with optic neuritis, transverse myelitis, or with brain lesions typical of NMO. Our three cases with NMO/NMOSD revealed five imaging features: (i) extensive transverse cord lesions, extending more than three vertebral segments, partially persisting as cavitation; (ii) periependymal lesions; (iii) lesions of the corticospinal tracts; (iv) extensive and confluent hemispheric white matter lesions reflecting vasogenic edema and partially involving the cerebral cortices and basal ganglia; and (v) two patterns of serial hemispheric white matter lesions: one is cavitation and another is partial regression or disappearance. Cavitations, in the upper spinal cord and hemispheric white matter, are considered to be caused by severe vasogenic edema and are likely to be one of the characteristic findings in NMOSD.

[A case of tuberculous myeloradiculitis with abdominal lymphadenitis presenting with symptoms of radiculomyelopathy].

A 30-year old man was admitted with right hip pain and gait disturbances. Neurological findings revealed muscular weakness in the lower limbs, hyporeflexia, dysesthesia in the sacral region, and bowel and bladder disturbances. Cerebrospinal fluid (CSF) examination indicated a white blood cell count of 371/µl (lymphocyte:polymorphonuclear leukocyte = 97:3), protein levels of 463 mg/dl and sugar of 20 mg/dl. Although CSF culture was negative, tuberculous infection was presumed. Magnetic resonance imaging revealed areas of enhancement in the intramedullary region surrounding the spinal cord and cauda equina. Enhanced computed tomography (CT) of the abdomen revealed lymph node swelling around the head of the pancreas. Biopsy of the lymph node swelling was culture-positive for Mycobacterium tuberculosis. Hence, assuming a diagnosis of tuberculous lymphadenitis of the abdomen, antitubercular drugs were started. Since antitubercular therapy had beneficial effects on the neurological symptoms and CSF findings, we diagnosed the patient with tuberculous myeloradiculitis. Systematic examinations including lymph node biopsy and cultures were useful for the diagnosis of tuberculous myeloradiculitis.

Plasma Brain Natriuretic Peptide is a Marker of Prognostic Functional Outcome in Non-Cardioembolic Infarction.

BACKGROUND: High plasma levels of brain natriuretic peptide (BNP) may also be observed in patients with non-cardioembolic infarction (CEI). We aimed to evaluate the relation between plasma BNP level, clinical parameters, and functional outcome in patients with and without CEI. METHOD: This study analyzed consecutive Japanese patients with acute ischemic stroke. Correlations between plasma BNP level and conventional risk factors for ischemic stroke were examined. Values of P less than .05 were considered statistically significant. RESULTS: This study analyzed 718 acute ischemic stroke patients (445 men and 273 women; mean age, 73.9 years). Mean plasma level of BNP was significantly higher for CEI (366.6 pg/ml) than for non-CEI (105.6 pg/ml; P < .01). Poor outcome (modified Rankin Scale score ≥3) at hospitalization and discharge were associated with significantly higher plasma BNP level than good outcome (modified Rankin Scale score ≤2) for both CEI and non-CEI. On multiple regression analysis, log-BNP was significantly associated with female sex, smoking, triglyceride, and creatinine clearance in CEI. In non-CEI, log-BNP was significantly associated with systolic/diastolic blood pressure, triglyceride, high-density lipoprotein cholesterol, and creatinine clearance. CONCLUSION: Irrespective of the presence of CEI, plasma BNP offers a marker of prognostic functional outcome. We clarified the characteristics and differences associated with plasma BNP in CEI and non-CEI, and our results suggest that plasma BNP can provide a useful marker of brain damage and neurohumoral dynamics in acute ischemic stroke.

Traumatic vertebral artery dissection and cerebral infarction following head and neck injury with a lucid interval.

Cases: Two patients with cerebral infarction following head and neck injury who showed a lucid interval are presented. Outcome: A 70-year-old male showed infarctions in the cerebellum bilaterally and the right hypothalamus on the sixth day after an injury with no fracture of the cervical spine, and bilateral dissection of the vertebral arteries was diagnosed. A 74-year-old male showed infarctions in the territory of the right posterior cerebral artery and posterior inferior cerebellar artery 2 days after injury with fractures of the cervical spine (C2 and C3) and was diagnosed as having artery-to-artery embolism based on dissection of the right vertebral artery. Conclusion: Head and neck injury is a very common presentation in the emergency department. Three-dimensional computed tomography angiography is an effective screening imaging method for vertebral artery dissection that should be carried out on arrival in every patient with fracture of the cervical spine, and even considered in doubtful cases with no fracture.

Primary Central Nervous System Lymphoma of the Cerebellopontine Angle That Initially Occurred as Neurolymphomatosis of the Acoustic Nerve.

We report a rare case of a primary central nervous system lymphoma (PCNSL) of the cerebellopontine angle (CPA) with infiltration into the pyramidal tract that initially presented as neurolymphomatosis (NL) of the acoustic nerve. A 60-year-old male suffered from right-side deafness and was referred to an otolaryngologist. Magnetic resonance imaging (MRI) showed fusiform enlargement of the right acoustic nerve with a hyperintense signal on a T2-weighted image (T2WI) and with gadolinium (Gd) enhancement, without an evidence of parenchymal CNS involvement. Although he was treated with steroids, his symptoms deteriorated. MRI was performed again and showed the mass lesion at the right CPA with enhancement. In addition to this, a lesion with slightly high intensity on a T2WI with Gd enhancement was observed along the right pyramidal tract. Despite steroid pulse therapy, the lesion rapidly progressed. We performed a tumor biopsy, and the histological diagnosis was diffuse large B-cell lymphoma. Pelvic, abdominal, and chest computed tomography scans, gallium cintigraphy, and bone marrow biopsy failed to detect any other evidence of lymphomatous involvement of other organs. We attempted high-dose methotrexate therapy (3.5 g/m2). We found a discrepancy in the therapeutic effect between the CPA lesion and the infiltrated lesion along the pyramidal tract; the lesions were chemo-resistant and chemo-sensitive, respectively. After completion of the second courses of chemotherapy, we began radiotherapy (total dose: 36 Gy). Four months after radiotherapy, the CPA tumor completely disappeared. Thirty-three months after the biopsy, he is doing well with a normal daily life and no signs of recurrence.

Emergency superficial temporal artery to middle cerebral artery bypass after intravenous recombinant tissue plasminogen activator administration for acute cerebral ischemia in a patient with moyamoya disease.

There are few study data to help in the decision whether to perform aggressive surgical revascularization, such as emergency bypass, after intravenous recombinant tissue plasminogen activator (rt-PA) administration in patients with progressive symptoms due to acute cerebral ischemia. A 33-year-old healthy male with no known previous medical history developed right hemiparesis and motor aphasia. No acute lesion was observed on admission computed tomography. According to the treatment protocol, emergency intravenous rt-PA administration was indicated within 3 h. After rt-PA administration, symptoms progressed to complete right hemiplegia. Emergency magnetic resonance imaging (MRI) showed an acute ischemic lesion in the left basal ganglia. MR angiography showed severe stenosis of the bilateral terminal portion of the internal carotid artery and occlusion of the left middle cerebral artery (MCA). Obvious diffusion-perfusion mismatch was detected. We performed digital subtraction angiography and diagnosed this condition as acute cerebral ischemia induced by moyamoya disease. We decided to perform emergency superficial temporal artery (STA)-MCA bypass to prevent further damage. The operation began 7 h after the administration of rt-PA and successful bypass was achieved. Symptoms stabilized and improved postoperatively. The majority of the area with preoperative hypoperfusion was rescued. Four months after surgery, the patient resumed his previous employment and continues to do well after 1.5 years of follow-up. This is the first report of emergency STA-MCA bypass performed after intravenous rt-PA administration for acute cerebral ischemia in a patient with moyamoya disease. We conclude that emergency STA-MCA bypass is a viable option for patients with moyamoya disease even after administration of rt-PA.

Heparin-induced thrombocytopenia in essential thrombocytosis.

We report a 67-year-old woman with essential thrombocytosis who developed cerebral infarction and heparin-induced thrombocytopenia during treatment for the cerebral infarction. She developed additional cerebral infarcts, acute femoral artery occlusion, and thrombophlebitis of her lower extremities. She was successfully treated with argatroban. This is the first report of a patient with essential thrombocytosis who developed heparin-induced thrombocytopenia and serious conditions, which included multiple thromboembolisms and coagulation disorders mimicking disseminated intravascular coagulation.

Moyamoya disease presenting as thalamic hemorrhage in a patient with neuromyelitis optica and Sjögren's syndrome.

A 52-year-old woman was admitted to the hospital with right thalamic hemorrhage. A carotid angiogram revealed occlusion of the terminal portions of the bilateral internal carotid arteries with basal moyamoya vessels, which was diagnosed as moyamoya disease (MMD). At 31 years of age, she was diagnosed with multiple sclerosis because of optic neuritis and myelitis. Paraplegia appeared 14 days after admission. T2-weighted thoracic magnetic resonance imaging revealed a high intensity lesion extending from T4 to T6. Her left upper limb was partially paralytic and her lower limbs exhibited paraplegia and dysesthesia. Anti-aquaporin 4 and anti-Sjögren's syndrome-A and -B antibodies were positive. The pathogenesis of neuromyelitis optica may be associated with such immunologic factors, but there are no reports of simultaneous presentations of neuromyelitis optica and MMD. Autoimmunity may be associated with the etiology of MMD.

Relationship between three-year survival and functional outcome at discharge from acute-care hospitals in each subtype of first-ever ischemic stroke patients.

BACKGROUND: We studied the relationship between survival prognosis and the functional outcome at discharge from acute-care hospitals in each subtype of ischemic stroke patients. METHODS: A total of 853 consecutive patients with first-ever ischemic stroke, recruited from December 1999 to November 2002, were followed for 725.8 ± 430.0 days. Functional outcome was scored using the modified Rankin scale (mRS). Survival analysis was performed using Kaplan-Meier curves, log rank test, and Cox regression model. RESULTS: The respective mortality of the groups with mRS scores of 0-2, 3, 4, and 5 were 13.1%, 25.0%, 47.2% (p<0.05), and 74.0% (p<0.05) in cardioembolic infarction (CEI) patients; 11.3%, 6.9%, 5.6%, and 46.7% (p<0.05) in atherothrombotic infarction (ATI) patients; 8.5%, 5.6%, 11.1%, and 0% (no patient) in lacunar infarction (LACI) patients; and 2.6%, 3.0%, 23.3% (p<0.05), and 28.9% (p<0.05) in infarction of unknown cause (IUC) patients. Multi-variate analysis showed that the mRS score was a significant predictive factor for death in CEI patients, and tended to be a predictive factor for death in ATI patients, or IUC patients. CONCLUSION: Functional outcome at discharge from acute-care hospitals may predict the survival prognosis of each subtype of ischemic stroke.

Purulent meningitis caused by Actinomyces successfully treated with rifampicin: a case report.

A 64-year-old woman presented with fever and headache. Lumbar puncture revealed cerebrospinal fluid (CSF) that contained 67,386 /mm(3) of WBC; CSF culture revealed Actinomyces species. She was diagnosed with purulent meningitis caused by actinomyces, and treated with intravenous ampicillin 12 g/day. The administration of ampicillin was effective, but not sufficient to control the inflammation in CSF. CSF inflammation persisted and a gradual increase in granulation tissue was found in the subdural space on lumbar MRI. After administration of rifampicin 450 mg/day, the CSF was normalized and the enhancement of granulation tissue decreased. The patient completely recovered 5 months after the therapy was initiated. We suggest that rifampicin may be an option for the treatment of meningitis caused by actinomyces.

Long-term prognosis of patients with large subcortical infarctions.

AIM: We assessed the long-term prognosis of patients with large subcortical infarctions (LSCI). METHODS: We defined LSCI as lesions > or =15 mm confined to deep penetrating arteries without a cardioembolic or atherothrombotic source. Patients with acute ischemic strokes were consecutively registered and followed for 751 +/- 441 days. The clinical characteristics and long-term prognoses of patients with LSCI were compared to those of patients with lacunar (LACI), atherothrombotic (ATI) and cardioembolic infarctions (CEI). RESULTS: At discharge from the hospital, the proportion of good outcomes (modified Rankin Scale < or =2) for patients with LSCI (52.1%) was similar to that for ATIs (47.2%), but worse than that for LACIs (73.2%). After a 3-year follow-up period, the mortality rates from LSCI, LACIs, ATIs and CEIs were 8.4, 8.2, 22.3 and 41.1%, respectively; the recurrence rates were 9.3, 14.1, 16.6 and 23.8%, respectively. CONCLUSIONS: The short-term prognosis of functional outcomes for LSCI was worse than that for LACIs, but similar to acute-phase ATI outcomes. The long-term prognosis after a LSCI is good, and recurrence tends to be lower than for LACIs.