Unusual Presentation, Common Disease

Abstract

A 65-year-old right-handed woman with a history of hypertension, remote tobacco use, depression, and anxiety presented to our hospital with acute onset of bilateral flinging arm movements and speech arrest witnessed by her boyfriend. She arrived at the emergency department (ED) within 1 hour of the symptom onset. On examination, she was hypertensive with a normal heart rate and temperature. She was awake and alert and only followed commands when asked to raise her legs. She mimicked eye closure and raised 2 fingers in both the hands. She had very little to no spontaneous speech and only answered questions with different random numbers throughout her stay in the ED. During attempts to name a pen or knuckles, she only verbalized numbers; reading and writing were not assessed. On cranial nerve examination, there was a slight right central facial nerve palsy. She moved all limbs equally against gravity. The flinging arm movements had resolved upon arrival at the ED. Her sensory examination was grossly normal as she withdrew her limbs to noxious stimulation, and her National Institutes of Health Stroke Scale (NIHSS) was 7. Noncontrast computed tomography (CT) of the head was unremarkable, and seizure was suspected because of the acute onset of arm movements for short duration and a possible postictal state with perseveration. However, CT angiography revealed a distal left M1 middle cerebral artery thrombus, and intravenous recombinant tissue plasminogen activator was administered. Subsequent brain magnetic resonance imaging (Figure 1) showed acute, patchy infarctions in the left basal ganglia, temporal, parietal, and frontal lobes, as well as a small left thalamic infarct. Over the next few days, the patient’s aphasic syndrome evolved into a mixed expressive and receptive aphasia, and she was discharged to rehabilitation for further speech therapy. Figure 1. Diffusion-weighted magnetic resonance imaging (MRI) image showing patchy restricted diffusion in the middle cerebral artery (MCA) and posterior cerebral artery (PCA) territories. The initial presentation of acute and transient bilateral motor activity suggested an epileptic event rather than stroke. However, only a small minority of strokes (0.3% in one study) present with seizure at onset.1 Alternatively, our patient’s bilateral arm movement may have been the result of infarction of the basal ganglia, though in this case only the left basal ganglia was affected. Although uncommon (about 1% in the Lausanne Stroke Registry), movement disorders (eg, ballism, dystonia, myoclonus, and tremor) can present acutely in the setting of stroke.2 A wide range of localizations have been cited including most commonly lesions of the basal ganglia and subthalamic nucleus. Other less common location of infarcts causing ballism include the thalamus, parieto-occipital areas and internal capsule, and areas involving both the anterior and the posterior circulation.3 Our patient’s aphasic syndrome consisted of perseveration only on different random numbers in response to all questions. Perseveration has long been a topic of interest to neurologists. In 1984, Sandson and Albert presented a new taxonomy of perseveration of speech: stuck-in-set, recurrent, and continuous. Stuck-in-set perseveration, defined as “inappropriate maintenance of a current category,” is seen in patients with dementia who, for instance, repeat numbers in the presented order when asked to repeat them backward. Continuous perseveration is the “inappropriate repetition, without interruption, of a current behavior.” This is exemplified in patients with subcortical injury.4 Recurrent perseveration is common in patients with aphasia caused by dominant hemispheric lesions. It is manifested by the repetition of a previous response after new stimulus is presented.5 Sandson and Albert later proposed that recurrent perseveration is based on left hemispheric temporal–parietal lesions, continuous perseveration is due to right hemispheric lesions, and stuck-in-set perseveration is due to frontosubcortical/mesolimbic lesions.4 Recent studies have noted perseveration resulting from lesions to the caudate head6 as well as the left supplementary motor area, basal ganglia, midbrain, and thalamus.7 Aphasias with sparing of number words have been described by Bencini et al. In their work, they studied a patient with left posterior temporal stroke who developed aphasia with error-free production of number words but with significant difficulty in uttering nonnumber words. They proposed linguistic reasons for the dissociation of number and nonnumber words, but a definitive mechanism and localization remain elusive.8 This case of patchy anterior and posterior circulation strokes demonstrates the sometimes unusual presentation of symptoms in patients with stroke. We hope this adds to the neurohospitalist’s understanding of acute vascular pathology.