Diana Apetauerova

Adverse effects of subthalamic nucleus DBS in a patient with multiple system atrophy

A 59-year-old woman with levodopa-responsive parkinsonism complicated by motor fluctuations and generalized levodopa dyskinesia underwent bilateral subthalamic deep brain stimulation (STN DBS) 7 years after symptom onset. DBS improved levodopa-responsive upper extremity bradykinesia but aggravated speech, swallowing, and gait. Motor fluctuations were not improved and levodopa dose remained unchanged. Pulse generators were turned off. Clinical features and brain MRI in this case were indicative of multiple system atrophy (MSA). STN DBS is not recommended for patients with MSA.

Successful bilateral deep brain stimulation of the globus pallidus internus for persistent status dystonicus and generalized chorea

The authors report the cases of 2 young male patients (aged 16 and 26 years) with dystonic cerebral palsy of unknown origin, who developed status dystonicus, an acute and persistent combination of generalized dystonia and chorea. Both patients developed status dystonicus after undergoing general anesthesia, and in 1 case, after administration of metoclopramide. In attempting to control this acute hyperkinetic movement disorder, multiple medication trials failed in both cases and patients required prolonged intubation and sedation with propofol. Bilateral deep brain stimulation of the globus pallidus internus (4 and 2 months after the onset of symptoms in the first and second case, respectively) produced immediate resolution of the hyperkinetic movement disorder in each case. Deep brain stimulation provided persistent suppression of the dystonic movement potential after a follow-up of 30 and 34 months, respectively, as demonstrated by the reemergence of severe dystonia during the end of battery life of the implantable pulse generators that was readily controlled by exchange of the generators in each case.

Motor cortex stimulation in patients with Parkinson disease: 12-month follow-up in 4 patients

OBJECT Since the initial 1991 report by Tsubokawa et al., stimulation of the M1 region of cortex has been used to treat chronic pain conditions and a variety of movement disorders. METHODS A Medline search of the literature published between 1991 and the beginning of 2007 revealed 459 cases in which motor cortex stimulation (MCS) was used. Of these, 72 were related to a movement disorder. More recently, up to 16 patients specifically with Parkinson disease were treated with MCS, and a variety of results were reported. In this report the authors describe 4 patients who were treated with extradural MCS. RESULTS Although there were benefits seen within the first 6 months in Unified Parkinsons Disease Rating Scale Part III scores (decreased by 60%), tremor was only modestly managed with MCS in this group, and most benefits seen initially were lost by the end of 12 months. CONCLUSIONS Although there have been some positive findings using MCS for Parkinson disease, a larger study may be needed to better determine if it should be pursued as an alternative surgical treatment to DBS.

Progression of Parkinson’s Disease following Thalamic Deep Brain Stimulation for Tremor

We assessed the long-term effect of thalamic deep brain stimulation (DBS) on motor symptoms and progression of Parkinson’s disease (PD) in PD patients treated for resting and postural/action tremor. Thalamic DBS was performed in 17 patients with treatment-resistant resting and postural/action tremor. Nine patients were available for follow-up examination a mean of 5.5 years after surgery. Three had tremor-dominant PD. DBS produced marked improvement in resting and postural/action tremor in target upper extremity in all 9 patients, which persisted unchanged at the time of the last follow-up visit 5.5 years after surgery. PD severity with DBS ‘on’ and ‘off’ 1 year after surgery was compared to PD severity at the last follow-up visit using UPDRS (Unified Parkinson’s Disease Rating Scale) III motor scores and individual motor item subscores. Patients were tested while on medication. There was no significant worsening of tremor, rigidity, speech, postural stability, gait, or axial bradykinesia with DBS either on or off at the last follow-up visit compared to the 12-month visit. UPDRS III motor scores were unchanged. However, global assessment of PD progression and increased mean L-dopa dose and L-dopa equivalent daily dose at the time of last follow-up visit indicated that a progression of PD had occurred.

CoQ10 in progressive supranuclear palsy A randomized, placebo-controlled, double-blind trial

Objective: An investigator-initiated, multicenter, randomized, placebo-controlled, double-blind clinical trial to determine whether coenzyme Q10 (CoQ10) is safe, well tolerated, and effective in slowing functional decline in progressive supranuclear palsy (PSP). Methods: Sixty-one participants received CoQ10 (2,400 mg/d) or placebo for up to 12 months. Progressive Supranuclear Palsy Rating Scale (PSPRS), Unified Parkinsons Disease Rating Scale, activities of daily living, Mini-Mental State Examination, the 39-item Parkinsons Disease Questionnaire, and 36-item Short Form Health Survey were monitored at baseline and months 3, 6, 9, and 12. The safety profile of CoQ10 was determined by adverse events, vital signs, and clinical laboratory values. Primary outcome measures were changes in PSPRS and Unified Parkinsons Disease Rating Scale scores from baseline to month 12. Results: CoQ10 was well tolerated. No statistically significant differences were noted between CoQ10 and placebo groups in primary or secondary outcome measures. A nonsignificant difference toward slower clinical decline in the CoQ10 group was observed in total PSPRS among those participants who completed the trial. Before the final study visit at 12 months, 41% of participants withdrew because of travel distance, lack of perceived benefit, comorbidities, or caregiver issues. Conclusions: High doses of CoQ10 did not significantly improve PSP symptoms or disease progression. The high withdrawal rate emphasizes the difficulty of conducting clinical trials in patients with PSP. ClinicalTrials.gov identifier: NCT00382824. Classification of evidence: This study provides Class II evidence that CoQ10 does not significantly slow functional decline in PSP. The study lacks the precision to exclude a moderate benefit of CoQ10.

Severe acute disseminated encephalomyelitis with normal MRI at presentation

Acute disseminated encephalomyelitis (ADEM) is a multifocal inflammatory demyelinating disease of the CNS with monophasic presentation, which often follows a recent illness or vaccination.1 In our review of the ADEM literature, white matter lesions are always evident on MRI scans. We report nearly complete recovery in a patient who developed coma due to severe ADEM, but in whom MRI abnormalities were not evident until several days after presentation. In May 1998, a 42-year-old woman experienced 6 days of cough, rhinorrhea, and diarrhea that were treated with cephalexin. On admission to another hospital, she had headache, stiff neck, fever (to 102 °F), intermittent confusion, and was unable to walk. Brain CT was normal. Lumbar puncture revealed 620 white cells/mm3 (84% neutrophils, 11% lymphocytes, 5% monocytes), 40 red cells/mm3, protein of 193 mg/dL, and glucose of 70 mg/dL. Gram stain was unrevealing. Ampicillin and ceftriaxone were started for presumed meningitis. Her medical history was unremarkable. There was no history of exposure to ticks, pets, travel outside …

Spinal Cord Stroke: Acute Imaging and Intervention

Spinal cord infarction is an uncommon disease and as such is often a diagnostic challenge for clinicians. It can vary in its onset, severity, outcome, and recovery from patient to patient. Treatment options for this relatively rare condition also remain elusive. Current consensus recommendations are antiplatelet therapy and the symptomatic management of associated complications such as paraplegia and thromboembolic disease. There are multiple studies in surgical literature of a variety of interventions and adjuncts used for reducing the risk of ischemic spinal cord neurological injury, seen most often in the setting of thoracoabdominal aortic repair operations. We report two cases of acute non-surgical-related spinal cord infarcts, where early diagnosis was made and aggressive, early treatments instituted. With often devastating outcomes, we highlight the need for early detection and that interventions, commonly used in preventing neurological injury after high-risk aneurysm repairs, may be beneficial in treating and reducing the severity of disability in acute spinal cord stroke.

Parkinson's disease: a motor control study using a wrist robot

Deep brain stimulation (DBS) is the most common surgical procedure for patients with Parkinsons disease (PD). DBS has been shown to have a positive effect on PD symptoms; however, its specific effects on motor control are not yet understood. We introduce the novel use of a wrist robot in studying the effects of stimulation on motor performance and learning. We present results from patients performing reaching movements in a null field and in a force field with and without stimulation. We discuss special cases where robotic testing reveals otherwise undiagnosed impairments, and where clinical scores and robot-based scores display opposing trends.

Reversible extrapontine and central pontine myelinolysis presenting with extrapyramidal features

ily history of neurological or movement disorder. The patient reported an overall improvement in his energy level and mood on the bupropion. However, he continued to complain of insomnia, poor concentration, and anxiety. Mirtazapine was added as augmentation for treatment of his residual insomnia, anxiety, and depressive symptoms. Shortly after the addition of mirtazapine at a dose of 15 mg/day, it was noted that his dyskinesia decreased in frequency and intensity. With further titration of the mirtazapine to 45 mg/day over the course of 4 weeks, the orofacial movements were no longer present. Bupropion is a novel antidepressant that has been postulated to function via dopaminergic pathways. There is a case report in the literature of buproprion-induced dyskinesia that reversed after discontinuation of the medication.1 The mechanism of action whereby buproprion induces dyskinesia may involve dopamine reuptake inhibition and the enhancement of norepinephrine functional activity.2 Mirtazapine is a novel antidepressant that disinhibits serotonergic and noradrenergic neurons by acting as an -2 antagonist. It also acts as a 5HT-2 and 5HT-3 antagonist and 5HT-1A agonist.3 Drugs with 5HT-1A agonistic activity (such as buspirone) and with 5HT-2 antagonistic activity (such as ritanserin) have been reported to be effective in reducing L-dopa–induced dyskinesia.4,5 There have also been case reports of mirtazapine being used to treat tremor and L-dopa–induced dyskinesia.6 A literature search revealed no case reports or studies of using mirtazapine to treat bupropion-induced dyskinesia. Patient management concerns did not allow us to explore direct causal relationships between bupropion and dyskinesias, or between mirtazapine and abatement of dyskinesias. Strict pharmacological testing would have included sequential withdrawal and reintroduction of the drugs to establish their specific roles. Nonetheless, to our knowledge, this case report is the first one to suggest that mirtazapine may be useful for the treatment of bupropion-induced dyskinesia in a similar manner to its reported efficacy in L-dopa–induced dyskinesia.

End of day dyskinesia in advanced Parkinson's disease can be eliminated by bilateral subthalamic nucleus or globus pallidus deep brain stimulation

We report the therapeutic effects of deep brain stimulation (DBS) in 2 patients with Parkinsons disease (PD) with severe end of dose dyskinesia that was resistant to medical therapy. In both patients, severe, end of day ballistic dyskinesias occurred when the last levodopa dose of the day was wearing off. Globus pallidus (GPi) DBS in 1 case and subthalamic (STN) DBS in the second case produced full resolution of end of day dyskinesia.