1.217 HEMIPARKINSONISM-HEMIATROPHY SYNDROME: NEUROIMAGING AND GENETICS

1.217 HEMIPARKINSONISM-HEMIATROPHY SYNDROME: NEUROIMAGING AND GENETICS

S50 Monday, 12 December 2011 / Parkinsonism and Related Disorders 18S2 (2012) S1–S79 Conclusion: Acute or subacute parkinsonism after striatal infar...

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Monday, 12 December 2011 / Parkinsonism and Related Disorders 18S2 (2012) S1–S79

Conclusion: Acute or subacute parkinsonism after striatal infarction can be diagnosed as vascular parkinsonism without great difficulty. However, in case with delayed appearance of parkinsonism several years after stroke, functional imaging such as DAT imaging is essential to differentiate superimposed degenerative parkinsonism. The concurrence of vascular lesion and DAT abnormality is more suggestive of vascular etiology in our case. 1.214 THROMBOEMBOLIC RISK AND THERAPY IN A PATIENT WITH PARKINSON’S DISEASE L. Thomas. Hematology/Oncology, The Regional Hospital, Scranton, PA, USA Introduction: Parkinson’s disease (PD) patients have an increased risk of death from pulmonary embolism (PE). The true incidence and cause of PE in PD patients is not clear. The decision to use Coumadin therapy, after addressing the thromboembolic events can necessitate complex decision-making. Case Presentation: An 89-year-old female with a history of PD was on Carbidopa/Levodopa. She reported shortness of breath for 10 days. Past history was negative for phlebitis, DVT or cancer. On examination, patient was noted to be tachypneic, tachycardic, and swelling in right leg. Ventilation/Perfusion scan showed multiple perfusion defects involving the right lung. High resolution CT scan confirmed PE. Venous duplex showed acute DVT’s in her right leg. Anticoagulation therapy was initiated with Heparin followed by Coumadin. The patient remained symptom free on Coumadin during the 10 months follow-up. The decision for prolonged anticoagulation was complicated by her life-threatening presentation despite the progressive PD status and increased fall risk. Discussion: PD is the most common neurodegenerative disorder leading to ambulatory dysfunction. These factors increase the risk of hypercoagulable predisposition. PE has been reported to be the second most common cause of death next to pneumonia in PD patients. Studies showed increased risk for asymptomatic DVT, especially when PD is severe. A causal relationship of Levodopa with thrombophilia has been hypothesized. The mechanism between PD and thromboembolic events remains unclear. Conclusion: The fall risk in PD patients is higher than the general population and the decision to evaluate the risks and benefits of Coumadin treatment becomes complex.

In conclusion, our findings suggest that FOG is associated with not only few structural lesions, but with wide disruption of neuronal circuits in brain and this disruption could be more sensitively detected by DTI than conventional MRI. 1.216 MOYAMOYA DISEASE INITIALLY MANIFESTING AS VASCULAR PARKINSONISM H.J. Cho1 , J.H. Choi1 , K.D. Choi1 , S.H. Kim2 . 1 Pusan National University Hospital, 2 Dong-A University Hospital, Busan, Republic of Korea Moyamoya disease is a cerebrovascular disorder characterized by progressive stenosis of the distal internal carotid artery, which can attribute to the wide range of clinical presentations. The majority of affected patients present with transient or fixed symptoms of cerebral ischemia such as hemiparesis, dysarthria and aphasia. However, vascular parkinsonism (VP) has been described as extremely rare clinical manifestation. We report a patient presenting with clinical symptom of VP as the initial manifestation of moyamoya disease. A 55-year-old woman presented with slowly progressive gait disturbance during a period of 2 years. She had no remarkable past history except for hypertension. On neurological examination, bradykinesia in all extremities, but more prominent on the left side, was noted with no resting tremor and rigidity. She showed smallstepped gait with pivotal turning, initiation failure and postural instability. Deep tendon reflexes were asymmetrically brisker on the left. T2-weighted and FLAIR images of the brain demonstrated diffuse hyperintensity involving cortical and subcortical areas of the bilateral frontal lobes. MRA and digital subtraction angiography of the brain revealed steno-occlusion at the terminal ends of the bilateral internal carotid arteries with the development of collateral vascular network. To our knowledge, this is the first case presenting with clinical symptoms of VP as the initial manifestation of moyamoya disease. Our case allows us to supplement clinical symptoms of VP to the initial manifestation of moyamoya disease, resulted from chronic ischemia to cortical and subcortical areas of the brain.

1.215 FREEZING OF GAIT IN PATIENTS WITH WHITE MATTER CHANGES IS RELATED WITH WIDE DISRUPTION OF WHITE MATTER J. Youn, J.W. Cho, H. Shin, J. Kim. Neurology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea

1.217 HEMIPARKINSONISM-HEMIATROPHY SYNDROME: NEUROIMAGING AND GENETICS E.S. Oh1 , J.-M. Kim1 , S.-S. Park2 , Y.K. Kim3 , S.E. Kim3 , S.-H. Oh4 , Z.-H. Cho4 . 1 Department of Neurology, Seoul National University Bundang Hospital, 2 Laboratory Medicine, 3 Nuclear Medicine, College of Medicine, National University, Seoul National University Bundang Hospital, Seoul, 4 Neuroscience Research Institute, Gachon University of Medicine and Science, Icheon, Republic of Korea

Freezing of gait (FOG) is a common and disabling symptom of parkinsonism. Even though FOG is generally considered to be related to the medial frontal lobe dysfunction, there are also studies about FOG and parietal, temporal or occipital lobe dysfunction. This implies that there should be different approach to explain FOG phenomenon. We performed diffusion tensor imaging (DTI) in 39 subjects with white matter changes detected by conventional MRI. We compared mean diffusivity (MD) and fraction anisotrophy (FA) values using tract-based spatial statistics between 14 subjects with FOG (FOG group) and 25 subjects without FOG (control group). There was no difference in sex, age, stroke risk factors and severity of white matter changes. FOG group demonstrate significantly low FA values in diffuse white matter tracts including both infratentorial structures such as pons, midbrain, cerebellar peduncle and cerebellum, and supratentorial structures such as frontal, parietal, temporal and occipital lobe except primary visual cortex. On the other hand, there was no difference in MD values between FOG and control groups.

Hemiparkinsonism-hemiatrophy (HP-HA) syndrome is characterized by early onset, slow progressive, asymmetric parkinsonism which is more prominent on the side of hemiatrophy. HP-HA syndrome is different from Parkinson disease in various ways but pathophysiology is unclear. We report a 40-year-old man who has HP-HA on his left side. He was born to monozygotic twins and denied birth related problems, head trauma history. He had no familial history of parkinsonism. He showed smaller face, shorter hand and foot on the left side than the right. Mild scoliosis and elevated left shoulder due to small sized trapezious muscle were found. His parkinsonian feature was prominent on the left; dystonia on the left shoulder but had mild rigidity in the right arm. The 3T MRI was nonspecific but on 7T MRI smaller, reduced signal hypodensity was observed in the right substantia nigra compared to the left side. 123 I-FP-CIT SPECT showed reduced uptake on the bilateral putamen on the right side. 18 FDG-PET showed globally decreased cortical binding and had no bilateral difference. [18 F]Flumazenil-PET revealed no significant difference, compared to 15 controls. We checked mutation in the parkin and heterozygous

Monday, 12 December 2011 / Parkinsonism and Related Disorders 18S2 (2012) S1–S79

duplication of exon 2 was detected. He showed good response to levodopa. Our understanding of the pathology of HP-HA syndrome are based on three kinds of factors: (1) perinatal or early cerebral insult, (2) disruption of the nigrostriatal system, and (3) genetic factors. The functional neuroimaging gives help to differential diagnosis of parkinsonism and prediction of the treatment effect as well as pathogenesis of HP-HA syndrome. 1.218 TWO CASES OF DURAL ARTERIOVENOUS FISTULA (DAVF) PRESENTING WITH PARKINSONISM AND PROGRESSIVE COGNITIVE DYSFUNCTION Y. Luo1,2 , J. Qi1 , W. Luo1 , H.T. Hu1 , B.R. Zhang1 . 1 Department of Neurology, the Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, 2 Department of Neurology, People’s Hospital of Qingtian, Qingtian, China We report two cases of dural arteriovenous fistula (DAVF) presenting as parkinsonism and progressive cognitive dysfunction which were misdiagnosed as Parkinson’s disease initially. Both cases had gradually progressive bradykinesia, gait disturbances and cognitive decline. One of them showed subcortical calcification on CT and confirmed as DAVF in the posterior fossa by DSA. And the other one accompanied with resting tremor and intracranial hypertension was also suggested as DAVF by MRA. They had poor or short-time response to the dopaminergic treatment. DAVF is a rare reason for patients with parkinsonism. This report emphasizes that we should be alert to the possibility of DAVF when we encounter the patients presenting with parkinsonism and progressive dementia, especially those with poor response to levodopa. 1.219 NEUROSYPHILIS PRESENTING WITH PARKINSONISM L. Fan, R. Yan. Department of Neurology, The First Affiliated Hospital of China Medical University, Shenyang, China Dear editor, Neurological manifestations of syphilis can and do occur during any stage of the infection. Although neurosyphilis is relatively common, case reports describing parkinsonism as a neurological complication following syphilis infection are limited. We report a case of parkinsonism noted on the half of the year of admission following dizziness, slow-walking and stolid reaction. Recent 2 weeks, he was found dysarthria, tremor, writing smaller characters, night hidrosis and constipation symptoms. 9 years ago, he had a history of feculent sexual contact and had genitalia indolent ulcer and condyloma acuminate. Physical examination on admission was found sialorrhea, monophony, repetitive words and dysdiadochokinesia. Myodynamia of limbs is III, and muscular tension of limbs is gear-like increase. We could see sporadic red papulas on the back, upper limbs and vulva. Laboratory examination showed that RPR is positive (+), titer is 1:32 and spirochaeta pallida (Tpa) antibody is 36.38 S/CO (normal <1.0). Etiology of CSF showed: Tpa antibody ELISA 0.679(+), TPPA(+). So we diagnosis him Parkinsonism complicating neurosyphilis. After the treatment of ceftriaxone and benzathine benzylpenicillin, association with Medopar, the patient had a good prognosis. Therefore, we should avoid missed diagnosis of neurosyphilis among the patients who had continuing unknown aggravated psychiatric, tremor, myotonia and such as parkinsonism-like symptoms. The fact that our patient responded well with levodopa suggests that neurosyphilis may have an effect on the striatal dopaminergic system. In conclusion, continuing parkinsonism as a sequelae of neurosyphilis should be recognised as a complication even after cholinergic symptoms have resolved.

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1.220 ¨ A PATIENT WITH SJOGREN SYNDROME WITH SYSTEMIC LUPUS ERYTHEMATOSUS AND TRIGEMINAL SENSORY NEUROPATHY AND PARKINSONISM (CASE REPORT) S. Nazarbaghi. Emam Khomeini Educational Center, Urmia University of Medical Sciences, Urmia, Iran Introduction: Sjogren ¨ syndrome is a slowly progressive autoimmune disease characterized by lymphocytic infiltration of exocrine glands resulting in Keratoconjunctivits sicca and xerostomia which may be combined with other Rheumatologic and Connective tissue Diseases. Neurologic Complications of this syndrome can be divided into neuromuscular manifestations occurring in 10–20% of Patients, and CNS Complications in 25% of Patients, most common of them is symmetric sensory polyneuropathy or Ganglionopathy of cranial nerves most commonly trigeminal nerve affected by sensory Ganglionopathy.parkinsonian features in SLE has been reported. Case report: The patient was a 45 years old woman with 2 years history of dry mouth and aphthous lesions of mouth and arthralgia with high titers of ANA and Anti ds DNA. Patient gradually developed severe sensory symptoms and dysesthesia of face and eye on right side. With decreased pinprick sensation of face.parkinsonism with masked face, tremor and cogwheel rigidity developed along with bradykinesia. Patient treated with prednisolon and Gabapentin (high dose) plus levodopa, Artan, amantadine with successful result. Discussion: Sjogren ¨ syndrome is one of important causes of sensory trigeminal neuropathy and should be considered for diagnosis of any patient presented by trigeminal nerve lesions.association of this syndrome and SLE presented here. 1.221 HASHIMOTO ENCEPHALOPATHY WITH PARKINSONISM F.N. Mercan, M.C. Akbostanci. Department of Neurology, Ankara University Medical Faculty, Ankara, Turkey Objective: To present a steroid-responsive case of parkinsonism and encephalopathy diagnosed to have Hashimoto encephalopathy (HE). Case was a 46-year-old male with sleep abnormalities, clumsiness in hands, behavioral changes and irritability. He had a diagnosis of goitre. Neurologic examination revealed mild bradykinesia and rigidity in the upper and lower extremities, symmetric bilateral action and intention tremor, hypomimia and parkinsonian gait. He also had neuropsychiatric symptoms such as disorientation in time and place, agitation, agressive behaviour and delusions of persecution. MMSE score was 23/30. EEG was normal. Hematological tests and complete rheumatologic panel were normal except mildly elevated serum C-reactive protein. Tumor markers were negative and serum ACE was normal. T2 weighted brain MRI showed frontal, parietal and occipital subcortical hyperintensity without enhancement. CSF protein (119 mg/dl), IgG (116 mg/dl) and albumin (774 mg/dl) were high. CSF ACE (8.70 I/U) was increased. Thoracic computed tomography (CT) was performed to rule out neurosarcoidosis. Serum TPO-Ab and TG-Ab were markedly increased (969 IU/mL, 1970.6 IU/mL respectively). Extensive microbiological tests on serum and CSF were negative. With a diagnosis of HE methylprednisolon of 1000 mg was given for a five days, then prednisolone 60 mg/day p.o was prescribed. His MMSE score was increased to 29/30 and his neurological and psychiatric symptoms vanished within two months of treatment. Discussion: HE is a condition that is life-threatening but easyto-diagnose with appropriate laboratory tests and treatable. This phenomenon should be considered in patients with subacute parkinsonism and neuropsychiatric symptoms.