Hesham Abboud

Impact of mild cognitive impairment on outcome following deep brain stimulation surgery for Parkinson's disease.

INTRODUCTION Unlike dementia, the effect of mild cognitive impairment (MCI) on outcomes after deep brain stimulation (DBS) in Parkinsons disease (PD) is less clear. We aimed to examine the effect of MCI on short- and long-term DBS outcomes. METHODS To study the effect of MCI type, cognitive domains (attention, language, visuospatial, memory, executive function), and Dementia Rating Scale (DRS) score on immediate postoperative outcomes (postoperative confusion, hospitalization days), PD patients who underwent DBS at our Center from 2006 to 2011 were analyzed. To determine cognitive predictors of intermediate (6-month) and long-term (1-year) post-operative outcomes, the changes in functional and quality-of-life (QOL) scores were analyzed in a smaller group with available preoperative health status measures. RESULTS We identified 130 patients [71% male, mean age: 63 ± 9.1, mean PD duration: 10.7 ± 5.1]. At preoperative assessment, 60% of patients had multiple-domain MCI, 21% had single-domain MCI, and 19% had normal cognition. MCI presence and type as well as DRS performance did not affect immediate outcomes. Attention impairment predicted longer postoperative hospitalization (P = 0.0015) and showed a trend towards occurrence of postoperative confusion (P = 0.089). For intermediate and long-term outcomes we identified 56 patients [73.2% male, mean age: 61.3 ± 9.6, mean PD duration: 10.6 ± 4.7]. Visuospatial impairment showed a trend towards less improvement in 6-month functional score (P = 0.0652), and 1-year QOL score (P = 0.0517). CONCLUSION The presence of MCI did not affect DBS outcomes. However, the types of impaired domains were more detrimental. Detailed cognitive testing can help stratify low- and high-risk patients based on their pattern of cognitive dysfunction.

Comprehensive, Multidisciplinary Deep Brain Stimulation Screening for Parkinson Patients: No Room for “Short Cuts”

Careful, often cumbersome, screening is a fundamental part of DBS evaluation in Parkinsons disease (PD). It often involves a brain MRI, neuropsychological testing, neurological, surgical, and psychiatric evaluation, and “ON/OFF” motor testing. Given that DBS has now been a standard treatment for advanced PD, with clinicians’ improved comfort and confidence in screening and referring patients for DBS, we wondered whether we can now streamline our lengthy evaluation process. We reviewed all PD patients evaluated for DBS at our center between 2006 and 2011 and analyzed the reasons for exclusion and for dropping out despite passing the screening process. A total of 223 PD patients who underwent DBS evaluation had complete charting. Only 131 (58.7%) patients were successfully implanted. Sixty‐one (27.3%) patients were excluded after screening because of significant cognitive decline (32.7%), early disease with room for medication adjustment (29.5%), behavioral dysfunction (21.3%), suspected secondary parkinsonism or atypical parkinsonism syndrome (13.1%), PD, but with poor levodopa response (11.4%), unrealistic goals (9.8%), PD with predominant axial symptoms (6.5%), significant comorbidities (6.5%), or abnormal brain imaging (3.2%). In addition, 31 (13.9%) patients were cleared for surgery, but either chose not have it (18 patients), were lost to follow‐up (12 patients), or were denied by medical insurance (1 patient). Through careful screening, a significant percentage of surgical candidates continue to be identified as less suitable because of a variety of reasons. This underscores the continued need for a comprehensive, multidisciplinary screening process.

Socioeconomic Status May Impact Functional Outcome of Deep Brain Stimulation Surgery in Parkinson's Disease

To investigate the association between socioeconomic status and deep brain stimulation (DBS) outcomes in Parkinsons disease (PD).

Camptocormia and Pisa syndrome as manifestations of acute myasthenia gravis exacerbation.

Camptocormia refers to abnormal forward flexion of the trunk that disappears with lying down [1]. Pisa syndrome is a related condition characterized by truncal lateral flexion [2]. Both considered a form of axial dystonia commonly seen in parkinsonism [3], dopamineblockers exposure [4], or as isolated movement disorders [5]. Camptocormia has been described in neuromuscular disorders secondary to paraspinal myopathy [6]. A few case reports described acute camptocormia in myasthenia gravis (MG) [7]. We herein describe a patient who presented with complex postural change in the setting of MG relapse. An 80-year-old male with 6-year-history of seropositive, EMGconfirmed MG presented with 5 weeks of dysphagia, hoarseness, and facial weakness. In addition, he developed a subacute posture change characterized by stooping forward and inclination to the right. His posture worsened with physical exertion and towards the end of the day. By bedtime, he described having to drag his right arm on the floor while walking. He had two previous relapses manifesting with bulbar/facial weakness but never with postural changes. He had been in remission off all MG medications for the past 3 years and had no prior exposure to dopamine-blockers. On examination, he had bilateral ptosis, facial weakness, mild dysarthria, nasal tone of voice, fatigable proximalmuscle weakness, and both camptocormia and Pisa syndrome to the right. His posture improved to near normal when lying down. There were no signs of parkinsonism on exam. Trial of oral pyridostigmine and low dose oral steroids as an outpatient did not help his symptoms. He eventually presented to the ED after he developed difficulty clearing his own secretions and as his posture continued to worsen. Given his history of prostate cancer, there were concerns for possible lumbar metastasis for which lumbar X-ray andMRI were done. Both studieswere negative for evidence ofmetastasis but did showmild levoscoliosis that could not explain his new and fluctuating postural change especially with the observed degree of severity. Being on anticoagulation for chronic atrial fibrillation, immediate plasma exchange was not possible and he was subsequently treated with 5 days of intravenous immunoglobulins (IVIG) plus oral steroids. His cranial/bulbar symptoms improved markedly but with only moderate posture improvement. One month later, and after IVIG booster as an outpatient, his posture improved significantly except for slight residual

Predictors of Functional and Quality of Life Outcomes following Deep Brain Stimulation Surgery in Parkinson’s Disease Patients: Disease, Patient, and Surgical Factors

Objective The primary objective was to evaluate predictors of quality of life (QOL) and functional outcomes following deep brain stimulation (DBS) in Parkinsons disease (PD) patients. The secondary objective was to identify predictors of global improvement. Methods PD patients who underwent DBS at our Center from 2006 to 2011 were evaluated by chart review and email/phone survey. Postoperative UPDRS II and EQ-5D were analyzed using simple linear regression adjusting for preoperative score. For global outcomes, we utilized the Patient Global Impression of Change Scale (PGIS) and the Clinician Global Impression of Change Scale (CGIS). Results There were 130 patients in the dataset. Preoperative and postoperative UPDRS II and EQ-5D were available for 45 patients, PGIS for 67 patients, and CGIS for 116 patients. Patients with falls/postural instability had 6-month functional scores and 1-year QOL scores that were significantly worse than patients without falls/postural instability. For every 1-point increase in preoperative UPDRS III and for every 1-unit increase in body mass index (BMI), the 6-month functional scores significantly worsened. Patients with tremors, without dyskinesia, and without gait-freezing were more likely to have “much” or “very much” improved CGIS. Conclusions Presence of postural instability, high BMI, and worse baseline motor scores were the greatest predictors of poorer functional and QOL outcomes after DBS.

Spinal Movement Disorders in Neuromyelitis Optica: An Under‐recognized Phenomenon

Spinal cord demyelination can cause several movement disorders. Although these abnormal movements could be the presenting symptom of the disease and, at times, the major source of disability, they are often overlooked, mislabeled, or undertreated. The aims of this study were to clearly define and establish common terminology for spinal movement disorders (SMDs) and characterize their full spectrum in patients with neuromyelitis optica (NMO).

Teaching NeuroImages: Manganese neurotoxicity of the basal ganglia and thalamus

A 27-year-old man with cholestasis presented with 4 weeks of progressive spastic quadriparesis and hypophonia. MRI revealed T2/fluid-attenuated inversion recovery hyperintensities in the basal ganglia and thalamus (figure 1). He was treated with IV methylprednisolone for acute disseminated encephalomyelitis. His condition worsened. MRI 4 weeks later showed larger symmetric hyperintensities in the basal ganglia and thalami (figure 2). Blood manganese was elevated (25.4 µg/L, reference 4.4–15.2). There was no known exposure. He was treated with chelation and levodopa with limited response.

Occupational Oromandibular Dystonia in an Opera Singer Mimicking Spasmodic Dysphonia

While occupational dystonia among singers is often described as laryngeal in nature, we describe a unique case of oromandibular (jaw closure/jaw deviation) dystonia in a professional opera singer occurring exclusively when attempting to sing in the higher registry. Occurring in the context of a high-risk profession, the patient’s tone-selective symptoms were mistaken for spasmodic dysphonia which obscured the correct diagnosis and resulted in delay of treatment. After establishing the diagnosis, significant improvement was noted with botulinum toxin injections to the mandibular muscles.