- Email: [email protected]
PO13.2 Botulinum Toxin Injections in Medically-Refractory Post-Stroke Holmes' Tremor
2009 Asian and Oceanian Congress of Clinical Neurophysiology
PO13. Movement Disorders PO13.1 Progressive Encephalomyelitis with Rigidity and Myoclonus: A Case Report Woong-Woo Lee1 *, Yoon-Ho Hong2 Dept. of Neurology, Seoul National University Hospital, Korea, 2 Dept. of Neurology, Seoul National University Boramae Hospital, Korea E-mail address: [email protected]
1
Background: Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a rare disorder which is characterized by brainstem signs, myoclonus, and hyperekplexia as well as progressive rigidity on axial and proximal limb muscles. We report a PERM patient, whose initial manifestation was isolated dysphagia followed several months later by rigidity and myoclonus. Case report: A 66-year-old female complained of dysphagia, progressed gradually through 2 weeks. Four weeks later, she developed bilateral conjugate gaze palsies without other neurological deficits. Intensive work-ups are including brain MRI, EMG, CSF study, viral serology test, tumor markers, antibody screening for autoimmune disorders, and anti-GM1/antiGQ1 antibody test, revealed no remarkable findings except for weakly positive serum anti-GQ1b IgM antibody. With intravenous immunoglobulin (IVIG) and oral corticosteroid treatment, she recovered completely one month later. One week after remission, she complained of stiffness and myoclonus in the left upper and both lower limbs, which were followed by conjugate gaze palsies again. Brain MRI revealed neither relevant findings nor interval changes, and anti-GQ1b and GAD antibody were all negative. On EMG study, there was no evidence of myotonia or neuromyotonia. With IVIG and oral corticosteroid treatment, all symptoms were improved. Since then, she has suffered from two more attacks for 1 year, which consisted of dysphagia, diplopia, stiffness, spasm and myoclonus. Conclusions: Symmetric rigidity and stiffness are initial symptoms in the majority of reported cases with PERM. It was unusual that the initial presentation was only the brainstem sign, and that the stiffness and myoclonus followed several months later. PERM could mimic autoimmune brainstem encephalitis like Bickerstaff encephalitis or Miller-Fisher syndrome, and might be associated with anti-GQ1b antibody. PO13.2 Botulinum Toxin Injections in Medically-Refractory Post-Stroke Holmes’ Tremor William S. Baek * Dept. of Neurology, Southern California Permanente Medical Group, USA E-mail address: [email protected] Background: Botulinum toxin type A is becoming the panacea of the 21st century, with FDA-approved indications in strabismus, blepharospasm, cervical dystonia, brow furrow, and axillary hyperhidrosis. Holmes’ tremor, also known as ‘rubral tremors’, does not respond well to medications traditionally used for tremors. We report a case of using Botulinum toxin injections in treating post-stroke Holmes’ tremor. Case report: A 58 year old male with mild hypertension sustained a small focal right cerebral peduncular ischemic stroke 3 years ago. Since then he has had bothersome left arm, leg and chin resting crescendo-decrescendo tremors, which would come in clusters. He needed to hold down his left arm at times. The tremors were worse at night affecting sleep and with stress. This required changing hands when holding papers, but did not affect tasks such as eating, dressing, or walking. On examination there were resting tremors in left chin, left cheek and left foot manifesting as dorsiflexion/plantar flexion and ankle rotation, left wrist flexion/ extension, forearm pronation/supination, finger abduction, with almost pill-rolling quality in fingers with DIP joint flexion/extension, provoked by distraction (video clip). There was intermittent L shoulder elevation. The rest of his exam was normal, including strength and tone. Lipid panel, homocysteine levels, hemoglobin A1c, anti-phospholipid antibody levels were all normal or negtiave. Carotid ultrasound and transthoracic echocardiogram were both normal. Brain MRI demonstrated a punctuate zone of T2 prolongation within the right cerebral peduncle (poster figure). Klonazepam 0.5 mg twice daily resulted in mild subjective improvement. Primidone 50 mg daily was added, initially decreasing tremors by 50% without sustained response. Levodopa-carbidopa 25/100 three times daily
S83 as needed was added, allowing mild objective improvement. EMG-guided Botulinum toxin type A injections were administered 2.5 years poststroke, every 3 months, starting at a total of 75 units, increasing up to 160 units as a 1 cc dilution. Injection sites included left mentalis, inferior orbicularis oris, pronator teres, flexor carpi radialis, adductor pollicis, flexor digitorum superficialis, flexor pollicis longus, pectoralis major, and extensor digitorum longus. Botulinum toxin injections resulted in complete resolution of facial tremors, near-complete resolution of hand tremors and a symptom-free period of 1.5 months in leg tremors. Conclusions: EMG-guided Botulinum toxin injections should be considered as a treatment modality in medically-refractory post-stroke Holmes’ tremor. PO13.3 Hypomania Induced by Subthalamic Nucleus Stimulation in a Parkinson’s Disease Patient A Case Report Ji-Seon Kim1 *, Sun Ha Paek2 , Beom S. Jeon3 1 Dept. of Neurology, Seoul National University, Bundang Hospital, Korea, 2 Dept. of Neurosurgery, Seoul National University, Korea, 3 Dept. of Neurology, Seoul National University, Korea E-mail address: [email protected] Background: STN-DBS is an established therapy for Parkinson’s disease. Mood changes, such as mania or depression, have been reported after surgery. We report a patient who developed hypomania induced by STN-DBS. Case report: A 59-year-old man with a 15-year history of PD underwent bilateral implantation of the electrodes to the STN. (Right DBS setting was 3.0 V of amplitude, 60 usec of pulse width, 130 Hz of frequency rate and left side was 2.2 V, 60 ms and 130 Hz.) He had no history of psychiatric problems, such as depression or mood disorders. Immediately after surgery his motor function was markedly improved and his mood was elevated excessively to the degree of hypomania. Hypomania slowly regressed over the several months. He continues to be euphoric to a mild degree at the time of 2 year follow-up visit. He is off medication and enjoys near normal motor function (UPDRS part III score 4). Fusion images of the preoperative MRI and postoperative CT scan showed that the electrodes were located in the medial portion of the STN, which has been reported to be associated with mood changes. Conclusions: This case supports the previous reports that hypomania can occur with stimulation of the medial part of the STN. PO13.4 The Wide Clinical Spectrum and Nigrostriatal Dopaminergic Damage in Spinocerebellar Ataxia Type 6 Jong-Min Kim1 *, Jee-Young Lee1 , Hee Jin Kim1 , Ji Sun Kim1 , Yu Kyeong Kim3 , Sung Sup Park2 , Sang Eun Kim3 , Beom Seok Jeon1 1 Dept. of Neurology, Seoul National University Hospital and Bundang Hospital, Korea, 2 Laboratory Medicine, Seoul National University Hospital and Bundang Hospital, Korea, 3 Nuclear Medicine, Seoul National University Hospital and Bundang Hospital, Korea E-mail address: [email protected] Background: Spinocerebellar ataxia type 6 (SCA6) manifests a widespectrum of non-cerebellar system involvements, including parkinsonism. However, the cause of parkinsonism is unknown. The aim of this study was to examine the presence of nigrostriatal dopaminergic system derangement in SCA6. Methods: Eight patients with SCA6 who underwent a regular follow-up participated in this study. Results: The main clinical feature of SCA6 was cerebellar ataxia with impaired eye movements. However, a wide-spectrum of non-cerebellar system involvements, such as dysfunctions of the autonomic nervous system, pyramidal and extrapyramidal signs, was also observed. In two patients, mild bradykinesia was noted, and one of these patients was administered L-dopa without benefit. [99mTc]-TRODAT-1 SPECT showed that in a patient manifesting mild bradykinesia (CAG repeats 13/22), DAT density was reduced to the Parkinson disease (PD) range with a rostrocaudal gradient typical of PD. The other patient with bradykinesia (12/25) had mildly decreased [99mTc]-TRODAT-1 uptake. Of the four patients without extrapyramidal signs, three (12/22, 11/25, 17/22) showed mild to severe reduction of DAT density, and one (13/22) had a normal binding. Conclusions: This study shows that SCA6 has a heterogeneous degree of the nigrostriatal dopaminergic system derangement. Two patients manifested mild bradykinesia, emphasizing the need to screen SCA6
PO13. Movement Disorders PO13.1 Progressive Encephalomyelitis with Rigidity and Myoclonus: A Case Report Woong-Woo Lee1 *, Yoon-Ho Hong2 Dept. of Neurology, Seoul National University Hospital, Korea, 2 Dept. of Neurology, Seoul National University Boramae Hospital, Korea E-mail address: [email protected]
1
Background: Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a rare disorder which is characterized by brainstem signs, myoclonus, and hyperekplexia as well as progressive rigidity on axial and proximal limb muscles. We report a PERM patient, whose initial manifestation was isolated dysphagia followed several months later by rigidity and myoclonus. Case report: A 66-year-old female complained of dysphagia, progressed gradually through 2 weeks. Four weeks later, she developed bilateral conjugate gaze palsies without other neurological deficits. Intensive work-ups are including brain MRI, EMG, CSF study, viral serology test, tumor markers, antibody screening for autoimmune disorders, and anti-GM1/antiGQ1 antibody test, revealed no remarkable findings except for weakly positive serum anti-GQ1b IgM antibody. With intravenous immunoglobulin (IVIG) and oral corticosteroid treatment, she recovered completely one month later. One week after remission, she complained of stiffness and myoclonus in the left upper and both lower limbs, which were followed by conjugate gaze palsies again. Brain MRI revealed neither relevant findings nor interval changes, and anti-GQ1b and GAD antibody were all negative. On EMG study, there was no evidence of myotonia or neuromyotonia. With IVIG and oral corticosteroid treatment, all symptoms were improved. Since then, she has suffered from two more attacks for 1 year, which consisted of dysphagia, diplopia, stiffness, spasm and myoclonus. Conclusions: Symmetric rigidity and stiffness are initial symptoms in the majority of reported cases with PERM. It was unusual that the initial presentation was only the brainstem sign, and that the stiffness and myoclonus followed several months later. PERM could mimic autoimmune brainstem encephalitis like Bickerstaff encephalitis or Miller-Fisher syndrome, and might be associated with anti-GQ1b antibody. PO13.2 Botulinum Toxin Injections in Medically-Refractory Post-Stroke Holmes’ Tremor William S. Baek * Dept. of Neurology, Southern California Permanente Medical Group, USA E-mail address: [email protected] Background: Botulinum toxin type A is becoming the panacea of the 21st century, with FDA-approved indications in strabismus, blepharospasm, cervical dystonia, brow furrow, and axillary hyperhidrosis. Holmes’ tremor, also known as ‘rubral tremors’, does not respond well to medications traditionally used for tremors. We report a case of using Botulinum toxin injections in treating post-stroke Holmes’ tremor. Case report: A 58 year old male with mild hypertension sustained a small focal right cerebral peduncular ischemic stroke 3 years ago. Since then he has had bothersome left arm, leg and chin resting crescendo-decrescendo tremors, which would come in clusters. He needed to hold down his left arm at times. The tremors were worse at night affecting sleep and with stress. This required changing hands when holding papers, but did not affect tasks such as eating, dressing, or walking. On examination there were resting tremors in left chin, left cheek and left foot manifesting as dorsiflexion/plantar flexion and ankle rotation, left wrist flexion/ extension, forearm pronation/supination, finger abduction, with almost pill-rolling quality in fingers with DIP joint flexion/extension, provoked by distraction (video clip). There was intermittent L shoulder elevation. The rest of his exam was normal, including strength and tone. Lipid panel, homocysteine levels, hemoglobin A1c, anti-phospholipid antibody levels were all normal or negtiave. Carotid ultrasound and transthoracic echocardiogram were both normal. Brain MRI demonstrated a punctuate zone of T2 prolongation within the right cerebral peduncle (poster figure). Klonazepam 0.5 mg twice daily resulted in mild subjective improvement. Primidone 50 mg daily was added, initially decreasing tremors by 50% without sustained response. Levodopa-carbidopa 25/100 three times daily
S83 as needed was added, allowing mild objective improvement. EMG-guided Botulinum toxin type A injections were administered 2.5 years poststroke, every 3 months, starting at a total of 75 units, increasing up to 160 units as a 1 cc dilution. Injection sites included left mentalis, inferior orbicularis oris, pronator teres, flexor carpi radialis, adductor pollicis, flexor digitorum superficialis, flexor pollicis longus, pectoralis major, and extensor digitorum longus. Botulinum toxin injections resulted in complete resolution of facial tremors, near-complete resolution of hand tremors and a symptom-free period of 1.5 months in leg tremors. Conclusions: EMG-guided Botulinum toxin injections should be considered as a treatment modality in medically-refractory post-stroke Holmes’ tremor. PO13.3 Hypomania Induced by Subthalamic Nucleus Stimulation in a Parkinson’s Disease Patient A Case Report Ji-Seon Kim1 *, Sun Ha Paek2 , Beom S. Jeon3 1 Dept. of Neurology, Seoul National University, Bundang Hospital, Korea, 2 Dept. of Neurosurgery, Seoul National University, Korea, 3 Dept. of Neurology, Seoul National University, Korea E-mail address: [email protected] Background: STN-DBS is an established therapy for Parkinson’s disease. Mood changes, such as mania or depression, have been reported after surgery. We report a patient who developed hypomania induced by STN-DBS. Case report: A 59-year-old man with a 15-year history of PD underwent bilateral implantation of the electrodes to the STN. (Right DBS setting was 3.0 V of amplitude, 60 usec of pulse width, 130 Hz of frequency rate and left side was 2.2 V, 60 ms and 130 Hz.) He had no history of psychiatric problems, such as depression or mood disorders. Immediately after surgery his motor function was markedly improved and his mood was elevated excessively to the degree of hypomania. Hypomania slowly regressed over the several months. He continues to be euphoric to a mild degree at the time of 2 year follow-up visit. He is off medication and enjoys near normal motor function (UPDRS part III score 4). Fusion images of the preoperative MRI and postoperative CT scan showed that the electrodes were located in the medial portion of the STN, which has been reported to be associated with mood changes. Conclusions: This case supports the previous reports that hypomania can occur with stimulation of the medial part of the STN. PO13.4 The Wide Clinical Spectrum and Nigrostriatal Dopaminergic Damage in Spinocerebellar Ataxia Type 6 Jong-Min Kim1 *, Jee-Young Lee1 , Hee Jin Kim1 , Ji Sun Kim1 , Yu Kyeong Kim3 , Sung Sup Park2 , Sang Eun Kim3 , Beom Seok Jeon1 1 Dept. of Neurology, Seoul National University Hospital and Bundang Hospital, Korea, 2 Laboratory Medicine, Seoul National University Hospital and Bundang Hospital, Korea, 3 Nuclear Medicine, Seoul National University Hospital and Bundang Hospital, Korea E-mail address: [email protected] Background: Spinocerebellar ataxia type 6 (SCA6) manifests a widespectrum of non-cerebellar system involvements, including parkinsonism. However, the cause of parkinsonism is unknown. The aim of this study was to examine the presence of nigrostriatal dopaminergic system derangement in SCA6. Methods: Eight patients with SCA6 who underwent a regular follow-up participated in this study. Results: The main clinical feature of SCA6 was cerebellar ataxia with impaired eye movements. However, a wide-spectrum of non-cerebellar system involvements, such as dysfunctions of the autonomic nervous system, pyramidal and extrapyramidal signs, was also observed. In two patients, mild bradykinesia was noted, and one of these patients was administered L-dopa without benefit. [99mTc]-TRODAT-1 SPECT showed that in a patient manifesting mild bradykinesia (CAG repeats 13/22), DAT density was reduced to the Parkinson disease (PD) range with a rostrocaudal gradient typical of PD. The other patient with bradykinesia (12/25) had mildly decreased [99mTc]-TRODAT-1 uptake. Of the four patients without extrapyramidal signs, three (12/22, 11/25, 17/22) showed mild to severe reduction of DAT density, and one (13/22) had a normal binding. Conclusions: This study shows that SCA6 has a heterogeneous degree of the nigrostriatal dopaminergic system derangement. Two patients manifested mild bradykinesia, emphasizing the need to screen SCA6