Entrapment of the temporal horn is an extremely rare pathologic condition occurring as a result o... more Entrapment of the temporal horn is an extremely rare pathologic condition occurring as a result of surgery for tumors, intraventricular infections, hemorrhage, or traumatic events involving the peritrigonal area. We report a case of a 58-year-old man who presented with pure Wernicke aphasia (never described before in the albeit rare cases of isolated temporal horn dilatation) that regressed completely following successful ventriculoperitoneal shunting. The relevant literature is also briefly reviewed.
Impaired facial expression, including spontaneous and emotional movements such as smiling, has be... more Impaired facial expression, including spontaneous and emotional movements such as smiling, has been often reported in Parkinson's disease (PD). There is a general consensus that spontaneous smiling is abnormal in PD. Investigations on posed smiling yield contrasting results. Moreover, no study has yet addressed the relationship between posed smiling and abnormalities of voluntary movements of the lower face, global motor impairment and the effects of dopaminergic medication. We investigated the kinematics of posed smiling (mimicking a smile shown in a picture) and those of voluntary movements of the lower face (showing the teeth as fast as possible - voluntary grinning) in 15 patients with PD (ON and OFF therapy) and in 16 healthy controls. Facial movements were recorded using a 3D optoelectronic system and analyzed using dedicated software. Some kinematic parameters of both posed smiling and voluntary grinning were abnormally lower in PD patients in comparison to healthy subjects. The kinematics of posed smiling correlated with those of voluntary grinning in PD patients but not in healthy controls. Posed smiling and voluntary grinning abnormalities were related to global motor severity but did not significantly improve upon L-dopa administration. These results suggest that posed smiling and voluntary grinning are both abnormal in PD patients and that they are likely mediated by a common pathophysiological mechanism.
Clinical studies in patients with MSA document facial motor abnormalities, but no studies have ob... more Clinical studies in patients with MSA document facial motor abnormalities, but no studies have objectively assessed blinking abnormalities in this condition. We enrolled patients diagnosed as having clinically probable MSA, 20 patients of the parkinsonian phenotype (MSA-P) and 10 patients of the cerebellar phenotype (MSA-C) and 20 healthy controls (HCs). Blinking was recorded with a three-dimensional optoelectronic motion system equipped with dedicated software for data analysis. During voluntary blinking, the interphase pause duration between the closing and opening phases lasted longer in MSA-P and in MSA-C patients than in HCs; the opening phase had increased duration and reduced peak velocity in MSA-P. During reflex blinking, the opening blink phase also lasted longer in MSA-P patients than in HCs. During spontaneous blinking, the rate and kinematics of the closing and opening blinking phases were lower in MSA-P and in MSA-C patients than in HCs. Blink reflex recovery was higher in patients than in HCs. No difference was found between MSA-P patients receiving or not receiving dopaminergic medication. Our objective assessment of upper facial movement documents abnormalities of voluntary, spontaneous, and reflex blinking in patients with MSA-P and MSA-C, reflecting cortical-basal ganglia and brainstem dysfunction.
Gilles de la Tourette syndrome is characterized by motor/vocal tics commonly associated with psyc... more Gilles de la Tourette syndrome is characterized by motor/vocal tics commonly associated with psychiatric disorders, including obsessive-compulsive disorder. We investigated primary motor cortex and brainstem plasticity in Tourette patients, exposed and unexposed to chronic drug treatment, with and without psychiatric disturbances. We also investigated primary motor cortex and brainstem plasticity in obsessive-compulsive disorder. We studied 20 Tourette patients with and without psychiatric disturbances, 15 with obsessive-compulsive disorder, and 20 healthy subjects. All groups included drug-naïve patients. We conditioned the left primary motor cortex with intermittent/continuous theta-burst stimulation and recorded motor evoked potentials. We conditioned the supraorbital nerve with facilitatory/inhibitory high-frequency stimulation and recorded the blink reflex late response area. In healthy subjects, intermittent theta-burst increased and continuous theta-burst stimulation decreased motor evoked potentials. Differently, intermittent theta-burst failed to increase and continuous theta-burst stimulation failed to decrease motor evoked potentials in Tourette patients, with and without psychiatric disturbances. In obsessive-compulsive disorder, intermittent/continuous theta-burst stimulation elicited normal responses. In healthy subjects and in subjects with obsessive-compulsive disorder, the blink reflex late response area increased after facilitatory high-frequency and decreased after inhibitory high-frequency stimulation. Conversely, in Tourette patients, with and without psychiatric disturbances, facilitatory/inhibitory high-frequency stimulation left the blink reflex late response area unchanged. Theta-burst and high-frequency stimulation elicited similar responses in drug-naïve and chronically treated patients. Tourette patients have reduced plasticity regardless of psychiatric disturbances. These findings suggest that abnormal plasticity contributes to the pathophysiology of Gilles de la Tourette syndrome. However, obsessive-compulsive disorder patients have normal cortical and brainstem plasticity.
We investigated primary motor cortex and brain stem plasticity in patients with Gilles de la Tour... more We investigated primary motor cortex and brain stem plasticity in patients with Gilles de la Tourette syndrome. The study group comprised 12 patients with Gilles de la Tourette syndrome and 24 healthy subjects. Patients were clinically evaluated using the Yale Global Tic Severity Scale. We tested cortical plasticity by conditioning left primary motor cortex with intermittent or continuous theta-burst stimulation in 2 separate sessions. Test stimulation consisted of 20 motor-evoked potentials recorded from right first interosseous muscle before and after theta-burst stimulation. We also tested brain stem plasticity by conditioning the right supraorbital nerve with facilitatory electric high-frequency stimulation delivered at the same time as the late response of the blink reflex or inhibitory high-frequency stimulation delivered before the late response on 2 separate sessions. Test stimulation consisted of 10 blink reflexes from the right orbicularis oculi muscle before and after high-frequency stimulation. After intermittent theta-burst stimulation, motor-evoked potential amplitudes in healthy subjects increased significantly but remained unchanged in patients. Similarly, after continuous theta-burst stimulation, motor-evoked potential amplitudes decreased significantly in healthy subjects but did not in patients. After facilitatory high-frequency stimulation, the blink reflex late response area in healthy subjects increased, whereas after inhibitory high-frequency stimulation, it decreased. Conversely, in patients, both interventions left the blink reflex late response area unchanged. The lack of the expected inhibitory and facilitatory changes in motor-evoked potential amplitudes and blink reflex late response area suggests that abnormal plasticity in the primary motor cortex and brain stem play a role in the pathophysiology of Gilles de la Tourette syndrome.
Sleep disorders are common in multiple system atrophy (MSA), but the prevalence of excessive dayt... more Sleep disorders are common in multiple system atrophy (MSA), but the prevalence of excessive daytime sleepiness (EDS) is not well known. To assess the frequency and associations of EDS in MSA. Survey of EDS in consecutive patients with MSA and comparison with patients with Parkinson disease (PD) and individuals without known neurologic disease. Twelve tertiary referral centers. Eighty-six consecutive patients with MSA; 86 patients with PD matched for age, sex, and Hoehn and Yahr stage; and 86 healthy subject individuals matched for age and sex. Epworth Sleepiness Scale (ESS), modified ESS, Sudden Onset of Sleep Scale, Tandberg Sleepiness Scale, Pittsburgh Sleep Quality Index, disease severity, dopaminergic treatment amount, and presence of restless legs syndrome. Mean (SD) ESS scores were comparable in MSA (7.72 [5.05]) and PD (8.23 [4.62]) but were higher than in healthy subjects (4.52 [2.98]) (P < .001). Excessive daytime sleepiness (ESS score >10) was present in 28% of patients with MSA, 29% of patients with PD, and 2% of healthy subjects (P < .001). In MSA, in contrast to PD, the amount of dopaminergic treatment was not correlated with EDS. Disease severity was weakly correlated with EDS in MSA and PD. Restless legs syndrome occurred in 28% of patients with MSA, 14% of patients with PD, and 7% of healthy subjects (P < .001). Multiple regression analysis (with 95% confidence intervals obtained using nonparametric bootstrapping) showed that sleep-disordered breathing and sleep efficiency predicted EDS in MSA and amount of dopaminergic treatment and presence of restless legs syndrome in PD. More than one-quarter of patients with MSA experience EDS, a frequency similar to that encountered in…
Entrapment of the temporal horn is an extremely rare pathologic condition occurring as a result o... more Entrapment of the temporal horn is an extremely rare pathologic condition occurring as a result of surgery for tumors, intraventricular infections, hemorrhage, or traumatic events involving the peritrigonal area. We report a case of a 58-year-old man who presented with pure Wernicke aphasia (never described before in the albeit rare cases of isolated temporal horn dilatation) that regressed completely following successful ventriculoperitoneal shunting. The relevant literature is also briefly reviewed.
Impaired facial expression, including spontaneous and emotional movements such as smiling, has be... more Impaired facial expression, including spontaneous and emotional movements such as smiling, has been often reported in Parkinson's disease (PD). There is a general consensus that spontaneous smiling is abnormal in PD. Investigations on posed smiling yield contrasting results. Moreover, no study has yet addressed the relationship between posed smiling and abnormalities of voluntary movements of the lower face, global motor impairment and the effects of dopaminergic medication. We investigated the kinematics of posed smiling (mimicking a smile shown in a picture) and those of voluntary movements of the lower face (showing the teeth as fast as possible - voluntary grinning) in 15 patients with PD (ON and OFF therapy) and in 16 healthy controls. Facial movements were recorded using a 3D optoelectronic system and analyzed using dedicated software. Some kinematic parameters of both posed smiling and voluntary grinning were abnormally lower in PD patients in comparison to healthy subjects. The kinematics of posed smiling correlated with those of voluntary grinning in PD patients but not in healthy controls. Posed smiling and voluntary grinning abnormalities were related to global motor severity but did not significantly improve upon L-dopa administration. These results suggest that posed smiling and voluntary grinning are both abnormal in PD patients and that they are likely mediated by a common pathophysiological mechanism.
Clinical studies in patients with MSA document facial motor abnormalities, but no studies have ob... more Clinical studies in patients with MSA document facial motor abnormalities, but no studies have objectively assessed blinking abnormalities in this condition. We enrolled patients diagnosed as having clinically probable MSA, 20 patients of the parkinsonian phenotype (MSA-P) and 10 patients of the cerebellar phenotype (MSA-C) and 20 healthy controls (HCs). Blinking was recorded with a three-dimensional optoelectronic motion system equipped with dedicated software for data analysis. During voluntary blinking, the interphase pause duration between the closing and opening phases lasted longer in MSA-P and in MSA-C patients than in HCs; the opening phase had increased duration and reduced peak velocity in MSA-P. During reflex blinking, the opening blink phase also lasted longer in MSA-P patients than in HCs. During spontaneous blinking, the rate and kinematics of the closing and opening blinking phases were lower in MSA-P and in MSA-C patients than in HCs. Blink reflex recovery was higher in patients than in HCs. No difference was found between MSA-P patients receiving or not receiving dopaminergic medication. Our objective assessment of upper facial movement documents abnormalities of voluntary, spontaneous, and reflex blinking in patients with MSA-P and MSA-C, reflecting cortical-basal ganglia and brainstem dysfunction.
Gilles de la Tourette syndrome is characterized by motor/vocal tics commonly associated with psyc... more Gilles de la Tourette syndrome is characterized by motor/vocal tics commonly associated with psychiatric disorders, including obsessive-compulsive disorder. We investigated primary motor cortex and brainstem plasticity in Tourette patients, exposed and unexposed to chronic drug treatment, with and without psychiatric disturbances. We also investigated primary motor cortex and brainstem plasticity in obsessive-compulsive disorder. We studied 20 Tourette patients with and without psychiatric disturbances, 15 with obsessive-compulsive disorder, and 20 healthy subjects. All groups included drug-naïve patients. We conditioned the left primary motor cortex with intermittent/continuous theta-burst stimulation and recorded motor evoked potentials. We conditioned the supraorbital nerve with facilitatory/inhibitory high-frequency stimulation and recorded the blink reflex late response area. In healthy subjects, intermittent theta-burst increased and continuous theta-burst stimulation decreased motor evoked potentials. Differently, intermittent theta-burst failed to increase and continuous theta-burst stimulation failed to decrease motor evoked potentials in Tourette patients, with and without psychiatric disturbances. In obsessive-compulsive disorder, intermittent/continuous theta-burst stimulation elicited normal responses. In healthy subjects and in subjects with obsessive-compulsive disorder, the blink reflex late response area increased after facilitatory high-frequency and decreased after inhibitory high-frequency stimulation. Conversely, in Tourette patients, with and without psychiatric disturbances, facilitatory/inhibitory high-frequency stimulation left the blink reflex late response area unchanged. Theta-burst and high-frequency stimulation elicited similar responses in drug-naïve and chronically treated patients. Tourette patients have reduced plasticity regardless of psychiatric disturbances. These findings suggest that abnormal plasticity contributes to the pathophysiology of Gilles de la Tourette syndrome. However, obsessive-compulsive disorder patients have normal cortical and brainstem plasticity.
We investigated primary motor cortex and brain stem plasticity in patients with Gilles de la Tour... more We investigated primary motor cortex and brain stem plasticity in patients with Gilles de la Tourette syndrome. The study group comprised 12 patients with Gilles de la Tourette syndrome and 24 healthy subjects. Patients were clinically evaluated using the Yale Global Tic Severity Scale. We tested cortical plasticity by conditioning left primary motor cortex with intermittent or continuous theta-burst stimulation in 2 separate sessions. Test stimulation consisted of 20 motor-evoked potentials recorded from right first interosseous muscle before and after theta-burst stimulation. We also tested brain stem plasticity by conditioning the right supraorbital nerve with facilitatory electric high-frequency stimulation delivered at the same time as the late response of the blink reflex or inhibitory high-frequency stimulation delivered before the late response on 2 separate sessions. Test stimulation consisted of 10 blink reflexes from the right orbicularis oculi muscle before and after high-frequency stimulation. After intermittent theta-burst stimulation, motor-evoked potential amplitudes in healthy subjects increased significantly but remained unchanged in patients. Similarly, after continuous theta-burst stimulation, motor-evoked potential amplitudes decreased significantly in healthy subjects but did not in patients. After facilitatory high-frequency stimulation, the blink reflex late response area in healthy subjects increased, whereas after inhibitory high-frequency stimulation, it decreased. Conversely, in patients, both interventions left the blink reflex late response area unchanged. The lack of the expected inhibitory and facilitatory changes in motor-evoked potential amplitudes and blink reflex late response area suggests that abnormal plasticity in the primary motor cortex and brain stem play a role in the pathophysiology of Gilles de la Tourette syndrome.
Sleep disorders are common in multiple system atrophy (MSA), but the prevalence of excessive dayt... more Sleep disorders are common in multiple system atrophy (MSA), but the prevalence of excessive daytime sleepiness (EDS) is not well known. To assess the frequency and associations of EDS in MSA. Survey of EDS in consecutive patients with MSA and comparison with patients with Parkinson disease (PD) and individuals without known neurologic disease. Twelve tertiary referral centers. Eighty-six consecutive patients with MSA; 86 patients with PD matched for age, sex, and Hoehn and Yahr stage; and 86 healthy subject individuals matched for age and sex. Epworth Sleepiness Scale (ESS), modified ESS, Sudden Onset of Sleep Scale, Tandberg Sleepiness Scale, Pittsburgh Sleep Quality Index, disease severity, dopaminergic treatment amount, and presence of restless legs syndrome. Mean (SD) ESS scores were comparable in MSA (7.72 [5.05]) and PD (8.23 [4.62]) but were higher than in healthy subjects (4.52 [2.98]) (P < .001). Excessive daytime sleepiness (ESS score >10) was present in 28% of patients with MSA, 29% of patients with PD, and 2% of healthy subjects (P < .001). In MSA, in contrast to PD, the amount of dopaminergic treatment was not correlated with EDS. Disease severity was weakly correlated with EDS in MSA and PD. Restless legs syndrome occurred in 28% of patients with MSA, 14% of patients with PD, and 7% of healthy subjects (P < .001). Multiple regression analysis (with 95% confidence intervals obtained using nonparametric bootstrapping) showed that sleep-disordered breathing and sleep efficiency predicted EDS in MSA and amount of dopaminergic treatment and presence of restless legs syndrome in PD. More than one-quarter of patients with MSA experience EDS, a frequency similar to that encountered in…
Uploads