Claudia Uribe Roca

Botulinum Toxin in a Case of Hemimasticatory Spasm With Severe Worsening During Pregnancy

Hemimasticatory spasm (HMS) is a rather uncommon movement disorder with a pathophysiology that is still unclear, although temporomasseter entrapment at the temporal fossa has been advanced. The authors present a case of HMS in a woman who experienced marked worsening in episode frequency and severity during pregnancy. Treatment with botulinum toxin led to marked improvement.

Vascular hemichorea/hemiballism and topiramate: Clinical/Scientific Notes

Although vascular hemichorea/hemiballism (HC/HB) has been reported to be self‐limited, in some cases, it can be irreversible and severely disabling. The standard treatment includes typical and atypical neuroleptics and GABA‐mimetic drugs. Topiramate is a new antiepileptic drug possessing a complex mechanism of action, including the enhancement of GABA‐mediated inhibition. We describe a 71‐year‐old patient with HC/HB who markedly improved after topiramate treatment.

Impulse control disorder and piribedil: report of 5 cases.

Recent studies suggest that impulse control disorders (ICD) in Parkinson disease are not uncommon, and antiparkinsonian therapy, mainly the use of dopaminergic agonists, plays a causal role in the development of these symptoms. Pramipexole has been mainly related to the occurrence of ICDs, although these disorders may occur when any dopaminergic agonist-based therapy is administered.In this paper, we describe 4 patients with Parkinson disease and 1 with multisystem atrophy who presented a history (several months or years) of pathological gambling, hypersexuality, punding, and pathological use of the Internet secondary to piribedil. This previously undescribed association suggests that the development of these disorders might be also related to piribedil administration. It is also the first report of a patient with multisystem atrophy developing such adverse effects.

Treatable inherited rare movement disorders

There are many rare movement disorders, and new ones are described every year. Because they are not well recognized, they often go undiagnosed for long periods of time. However, early diagnosis is becoming increasingly important. Rapid advances in our understanding of the biological mechanisms responsible for many rare disorders have enabled the development of specific treatments for some of them. Well‐known historical examples include Wilson disease and dopa‐responsive dystonia, for which specific and highly effective treatments have life‐altering effects. In recent years, similarly specific and effective treatments have been developed for more than 30 rare inherited movement disorders. These treatments include specific medications, dietary changes, avoidance or management of certain triggers, enzyme replacement therapy, and others. This list of treatable rare movement disorders is likely to grow during the next few years because a number of additional promising treatments are actively being developed or evaluated in clinical trials.

Analysis of D216H polymorphism in Argentinean patients with primary dystonia.

Abstract The D216H polymorphism (rs1801968) in TOR1A has been suggested as a risk factor for developing primary dystonia in German subjects not carrying the deletion c.904-906delGAG (∆GAG). However, this association could not be confirmed in other populations with different ethnic backgrounds. The purpose of this study is to evaluate the D216H polymorphism in an Argentinean cohort of 40 patients with primary dystonia and 200 unrelated control subjects. The authors could observe a significantly higher frequency of the H216 variant in dystonic patients lacking ∆GAG as compared with controls.

Levetiracetam-induced Parkinsonism in a Huntington disease patient.

To the Editor, We recently read the article by Zesiewicz et al, where the authors report on levetiracetam (LEV)-induced parkinsonism in a patient with Huntington disease (HD). We report a 36-year-old white man with a molecular diagnosis of HD who developed marked extrapyramidal rigidity and dysarthria during treatment with LEV. The initial symptoms began in 1997 and were characterized by anxiety, claustrophobia, and panic attack. Dystonic and choreiform movements appeared over the following years. In 2004, a molecular diagnosis of HD was performed, and an expansion of a trinucleotide CAG to a number of 46 repeats was identified in the abnormal allele. On admission to our institution, the patient was on olanzapine 10 mg/d, clonazepam 1 mg, amantadine 250 mg, paroxetine 20 mg/d, and coenzyme Q10 800 mg/d. General physical examination was normal. The Mini-Mental State Examination score was 27 of 30 points. The neurological examination showed an alert patient with moderate dysarthria. His eye movement examination disclosed difficulty generating saccades in the horizontal direction. Dystonic postures were present in both hands, and choreiform movements were observed in the trunk and lower limbs (Unified Huntington Disease Rating Scale [UHDRS] subscore chorea, 2). Motor impersistence was also evident. He was able to walk without assistance, but the tandem walking was impaired with more than 3 deviations. The motor assessment of UHDRS was 26 points, and the Hamilton Depression Rating Scale score was 7. Over the last years, several reports have suggested that LEV could be useful in the treatment of different movement disorders. In accordance with these observations, we started our patient on LEV 250 mg/d during the first week. The drug dose was titrated upward by 250 mg each subsequent week. At 500 mg/d, the chorea improved (UHDRS chorea subscore, 1), but the patient noticed mild dizziness and a more pronounced dysarthria. The motor UHDRS score was 23 and Hamilton score was 6. When the patient increased the dose to 500 mg twice a day, his wife reported severe dysarthria (he was unable to speak for 48 hours, and the UHDRS dysarthria subscore was 4). He simultaneously presented a marked axial and mandibular rigidity (UHDRS score was 3). However, the choreiform movements remained unchanged since the last examination. In addition, his wife reported sleep disorders characterized by somniloquy and abnormal movements involving the 4 limbs. Neither clinical nor metabolic disorders were identified, and no change in concomitant medication was observed. The LEV was tapered by 250 mg/d every 3 days until 250 mg/d was reached. Rigidity and dysarthria improved partially. We therefore reduced and maintained LEV at 125 mg/d. At present, the UHDRS dysarthria subscore is 1 and rigidity is 0; however, choreic movements have not worsened with respect to the baseline condition. As in the case by Zesiewicz et al, our patient developed extrapyramidal rigidity as a side effect induced by LEV. We agree with the authors that the probable pathophysiology of this side effect could be multifactorial. One of the mechanisms involved could be the interaction between drugs, such as olanzapine, paroxetine, and LEV. Another hypothesis is that the action of LEV at the synaptic vesicle protein SV2A may interfere with dopamine release. In summary, considering these 2 cases, an extensive, double-blind study is required to determine whether LEV is safe and useful for treating choreiform symptoms in HD.

BLOOD PRESSURE EVOLUTION IN YOUNG PATIENTS WITH ACUTE ISCHEMIC STROKE: A new model for understanding the natural course of spontaneous hypertension?

ABSTRACT Background: Approximately 80% of patients suffering an acute ischemic stroke develop transient hypertension. The physiopathological mechanism remains unclear. Due to the lack of vascular risk factors, young adults could be a useful model for understanding blood pressure (BP) evolution in this setting. Methods: Patients between 18 and 55 years old admitted with an acute ischemic stroke were included. BP was evaluated during the following 48 h. Hypertension was defined as: systolic blood pressure (SBP) ≥140 mmHg or diastolic blood pressure (DBP) ≥90 mmHg. Patients were divided into two groups: RF group included those who had a previous vascular risk factor and/or other medical condition known to affect the autonomic function; noRF group included patients without any of the previously stated conditions. Results: Thirty-nine patients were included: mean age: 44.26 years old, 21 were males (53.8%). Mean SBP and DBP at admission were 139.77 ± 5.35 mmHg (range: 90–243) and 84.44 ± 3.02 mmHg (range: 60–128), respectively; 43.58% patients were hypertensive at admission. SBP decreased significantly during the first 48 h (p = 0.044) for the entire population. RF group has higher SBP (p = 0.009) and DBP (p = 0.011) at admission than the noRF group. Conclusion: Most patients were normotensive at admission and BP fell spontaneously despite BP at admission. Young patients without medical conditions that could alter the autonomic system function could be a useful model for understanding acute hypertension in ischemic stroke.

RAPIDLY PROGRESSIVE DEMENTIA SECONDARY TO AUTOIMMUNE ENCEPHALITIS: NEUROPSYCHOLOGICAL PROFILES AND RESPONSE TO IMMUNOTHERAPY

P2-313 IS CUED RECALL OF SHARED AUTOBIOGRAPHICAL KNOWLEDGE USEFUL IN CLINICAL DEMENTIA ASSESSMENT? Claudia Frankenberg, Maren Knebel, Christina Degen, Nadeshda Andrejeva, Petra Wetzel, Lina Sidonija Gorenc-Mahmutaj, Sabrina Dominique Navratil, Inga Meyer-K€uhling, Britta Wendelstein, Johannes Schr€oder, Section of Geriatric Psychiatry, University Hospital Heidelberg, Heidelberg, Germany; Interdisciplinary Ageing Research, Faculty of Educational Sciences, Goethe University, Frankfurt am Main, Germany; Institute of Gerontology, University of Heidelberg, Heidelberg, Germany. Contact e-mail: Claudia.Frankenberg@med.uni-heidelberg.de