William G. Ondo

Stereotypies are frequently reported in frontotemporal dementia. Their pathophysiology and optimal treatment are largely unexplored. The author reports seven cases of marked stereotype in patients with probable frontotemporal dementia who were treated with tetrabenazine, a monoamine inhibitor. All patients had near-complete resolution of stereotypy. Six patients stopped tetrabenazine at some point, with return of their stereotype within 24 hours, which again improved in the four who restarted therapy. Efficacy has not waned over time. Tetrabenazine dramatically improved stereotypies in patients with dementia. This observation could increase understanding of this interesting phenotype.

Task-specific tremor diagnoses remain controversial. We evaluated 56 subjects seen with writing tremor. The diagnosis was made if there was a clear history of exclusive tremor while writing for at least 3 years before noticing tremor in any other scenario and the continued presence of writing tremor as the most prominent aspect of their tremor disorder on examination. The age of tremor onset was 47.2 ± 18.0 years (73.2% male). Ethnic backgrounds were Caucasian (68.4%), African (23.2%), Hispanic (5.2%), and Asian/Indian (3.3%), and 44% reported any tremor in a first degree relative. Writing tremor often progressed to other task-specific tremors or rest tremor but not to immediate postural tremor, as usually seen in essential tremor. The other tremor provoking scenarios were eating/drinking (14), brushing teeth/shaving/make-up (5), typing (2), suture removal (1), and drafting (1) and occurred a mean of 7.5 years after the onset of writing tremor. Fourteen developed a “rest” (true rest or crescendo) tremor but only 2 of these met clinical criteria for Parkinson's disease. Pharmacologic treatments of writing tremor, including with ethanol, were generally poor, whereas deep brain stimulation of the ventral intermediate (VIM) thalamus was successful. Compared with patients with “classic” essential tremor in our clinic, writing tremor patients were more likely African, more likely male, had an older age of onset, a lower likelihood of familial tremor, and were more refractory to tremor medications and ethanol. This supports segregation between task-specific tremor and essential tremor but does not support the specific diagnosis of “writing tremor” because many patients progress to tremor with other tasks.

Management of motor complications in advanced Parkinson's disease (PD) can be challenging. The main complications are inadequate dopaminergic tone (“off” time and dose failures) and excess dopaminergic tone (dyskinesia). These motor complications increase as PD progresses. Changing the dose and timing of L-dopa is the main strategy for both scenarios. Reducing “off” time can also be achieved by the addition of adjunctive therapies (dopamine agonists, catechol-O-methyl transferase inhibitors, and monoamine oxidase-B inhibitors). Dyskinesia can improve with amantadine and possibly several other medications. Surgical interventions such as lesioning and deep brain stimulation are considered when pharmacological strategies for motor complications are not satisfactory.

Obsessive–compulsive disorder (OCD) is a disabling neuropsychiatric disorder, which rarely spontaneously remits. We present a case of a patient suffering with severe OCD whose symptoms disappeared immediately following a small right posterior frontoparietal infarct. We speculate that the lesion modulated the cortical–subcortical circuits, likely through a change in input into in the dorsolateral prefrontal cortex. This could have implications regarding surgical targeting in OCD.

Levodopa use in fluctuating Parkinson's disease (PD) is complicated by an inconsistent and prolonged onset to clinical improvement. An orally dissolved carbidopa/levodopa (OD C/L) preparation (Parcopa UCB Pharma) is available in the United States. This offers potential advantages to shorten the duration from ingestion to clinical improvement. Surprisingly, this has never been clinically assessed. We tested 20 patients with fluctuating PD and a Unified Parkinson's Disease Rating Scale (UPDRS) “off” motor score of ≥25 in a 2‐day, single‐dose, double‐blind, double‐dummy, crossover study. Patients arrived in the morning in the practically defined “off” state and were randomly assigned to receive identical doses of either oral C/L and OD placebo or OD C/L and oral C/L placebo on 1st day and the reverse combination on a 2nd day. After training, patients underwent bilateral hand tapping at baseline and every 5 minutes for 60 minutes after dose ingestion. Stride length (SL) was recorded at 5‐minute intervals with an ambulatory gait monitor. Patients identified their subjective latency to “on” and noted drug preferences and adverse events. They also underwent a UPDRS motor examination at baseline and 60 minutes after dose. Twenty subjects [15 male, age 68.7 (9.7), PD duration 13.4 (6.8)] completed. There were no significant group differences in tapping speed, subjective time to “on,” latency of increased SL, or overall preference. However, all trends did favor OD C/L. Adverse events were similar. This small pilot study did not show significant group differences favoring OD C/L; however, larger studies may be justified, and individual patients may benefit. © 2010 Movement Disorder Society