- Email: [email protected]
Unilateral postural tremor caused by frontal cavernoma
Clinical Neurology and Neurosurgery 125 (2014) 237–238
Contents lists available at ScienceDirect
Clinical Neurology and Neurosurgery journal homepage: www.elsevier.com/locate/clineuro
Letter to the Editor Unilateral postural tremor caused by frontal cavernoma Keywords: Tremor Cavernoma MRI
A 39-year-old man was admitted for a progressive right arm tremor lasting for more than 15 years. He had no medical past history. We found no history of tremor in his family. He had no medication. Tremor had begun progressively when he was around 23-year-old. He only felt disability in personal activities of daily living, such as drinking and writing since one year. Clinical examination showed a postural right tremor affecting the hand, exacerbated by stress. Tremor was discreetly present at action but absent at rest (cf. video) Left arm was free of tremor. We found no tremor of the head or legs. The patient had no parkinsonian sign. We found no sign of cerebellar dysfunction at clinical examination. His voice was normal. Alcohol consumption did not reduce the tremor. General clinical examination was normal and found no clinical sign of hyperthyroidism. We hypothesized that this patient had essential tremor (ET). As the patient was younger than 40 years old, brain imaging was requested. Brain MRI (cf. Fig. 1) showed a rounded hypo-intensity on T2 echo gradient sequences in the left posterior cingulate gyrus under the paracentral lobule compatible with the diagnosis of unique cavernoma without evidence of acute hemorrhage. This unique lesion was graded as a type IV cavernoma according to Zabramski et al. [1] classification. Electrophysiologic analysis showed a postural rhythmic tremor at 8 Hz affecting the right arm. We proposed to introduce propranolol at low dose (80 mg per day) with a great effect. The cavernoma was neither removed nor irradiated. We report the case of a patient presenting an unilateral tremor revealing a left hemispheric cavernoma. Cavernoma may be responsible for a wide variety of movement disorders depending on their location within the central nervous system. Midbrain cavernoma can cause Holmes tremor by interrupting the cerebello-rubrothalamic pathway and/or palatal myoclonus following degeneration of the inferior olivary nucleus due to a primary lesion in the dento-rubro-olivary pathway [2]. Furthermore, Südmeyer et al. [3] have reported the case of a patient with a brainstem cavernoma who presented with an asymmetric action tremor mimicking ET. Finally, cavernoma of the basal ganglia may also be responsible for movement disorders such as hemi-chorea when located in the caudate nuclei, segmental hémidystonia and hemi-parkinsonism. Here, we present the case of a patient presenting with unilateral tremor revealing a left hemispheric cavernoma. We hypothesized that the cavernoma disrupts fibers linking motor areas to the thalamus, altering neural communication within this motor network, http://dx.doi.org/10.1016/j.clineuro.2014.07.033 0303-8467/© 2014 Elsevier B.V. All rights reserved.
Fig. 1. Brain MRI showing (a) a left rounded hypointensity on T2 echo gradient sequences in the posterior cingulate gyrus and (b) its view in sagital T1 sequences.
thus leading to the controlateral tremor. In fact, electrophysiological studies on ET have demonstrated that pre-motor cortex, primary motor cortex and thalamus are parts of the oscillating network involved in ET and are closely linked to the cerebellum and the brainstem [4]. Finally, we cannot exclude that tremor in our patient can be essential in nature and that the cavernoma is an incidental finding. In conclusion, our observation emphasizes that brain imaging is needed to explore tremor mimicking asymmetric essential tremor, particularly for young adults. Conflict of interest The authors declare no conflicts of interest. Authors contributions Romain Lefaucheur and David Maltête: acquisition of data, analysis and interpretation, critical revision of the manuscript for important intellectual content, study supervision. Stéphane Derrey, Axel Lebas, David Wallon: acquisition of data, analysis and interpretation. Acknowledgment We thank Dr A. Lebas for his help to create brain MRI figure with his software JimboDicomviewer. Appendix A. Supplementary data Supplementary material related to this article can be found, in the online version, at http://dx.doi.org/10.1016/j.clineuro. 2014.07.033.
238
Letter to the Editor / Clinical Neurology and Neurosurgery 125 (2014) 237–238
References [1] Zabramski JM, Wascher TM, Spetzler RF, Johnson B, Golfinos J, Drayer BP, et al. The natural history of familial cavernous malformations: results of an ongoing study. J Neurosurg 1994;80(3):422–32. [2] Maduri R, Barbagallo G, Iofrida G, Signorelli M, Signorellli F. Regression of Benedikt’s syndrome after single-stage removal of mesencephalic cavernoma and temporal meningioma: a case report. Clin Neurol Neurosurg 2013;115(6):748–50. [3] Südmeyer M, Maroof P, Saleh A, Hartmann C, Wojtecki L, Schnitzler A. Action tremor caused by olivary cavernoma in Klippel–Trénaunay syndrome mimicking asymmetric essential tremor. J Neurol 2011;258(1):140–2. [4] Schnitzler A, Münks C, Butz M, Timmermann L, Gross J. Synchronized brain network associated with essential tremor as revealed by magnetoencephalography. Mov Disord 2009;24(11):1629–35.
Romain Lefaucheur ∗ Department of Neurology, Rouen University Hospital and University of Rouen, Rouen Cedex, France Stéphane Derrey Department of Neurosurgery, Rouen University Hospital and University of Rouen, Rouen Cedex, France Axel Lebas Department of Neurophysiology, Rouen University Hospital and University of Rouen, Rouen Cedex, France
David Wallon Department of Neurology, Rouen University Hospital and University of Rouen, Rouen Cedex, France a
David Maltête a,b Department of Neurology, Rouen University Hospital and University of Rouen, Rouen Cedex, France b INSERM U614, Rouen Faculty of Medicine, Rouen Cedex, France ∗ Corresponding
author at: Department of Neurology, Rouen University Hospital, 76031 Rouen Cedex, France. Tel.: +33 2 32 88 87 40; fax: +33 2 32 88 87 41. E-mail address: [email protected] (R. Lefaucheur) 8 May 2014 Available online 7 August 2014
Contents lists available at ScienceDirect
Clinical Neurology and Neurosurgery journal homepage: www.elsevier.com/locate/clineuro
Letter to the Editor Unilateral postural tremor caused by frontal cavernoma Keywords: Tremor Cavernoma MRI
A 39-year-old man was admitted for a progressive right arm tremor lasting for more than 15 years. He had no medical past history. We found no history of tremor in his family. He had no medication. Tremor had begun progressively when he was around 23-year-old. He only felt disability in personal activities of daily living, such as drinking and writing since one year. Clinical examination showed a postural right tremor affecting the hand, exacerbated by stress. Tremor was discreetly present at action but absent at rest (cf. video) Left arm was free of tremor. We found no tremor of the head or legs. The patient had no parkinsonian sign. We found no sign of cerebellar dysfunction at clinical examination. His voice was normal. Alcohol consumption did not reduce the tremor. General clinical examination was normal and found no clinical sign of hyperthyroidism. We hypothesized that this patient had essential tremor (ET). As the patient was younger than 40 years old, brain imaging was requested. Brain MRI (cf. Fig. 1) showed a rounded hypo-intensity on T2 echo gradient sequences in the left posterior cingulate gyrus under the paracentral lobule compatible with the diagnosis of unique cavernoma without evidence of acute hemorrhage. This unique lesion was graded as a type IV cavernoma according to Zabramski et al. [1] classification. Electrophysiologic analysis showed a postural rhythmic tremor at 8 Hz affecting the right arm. We proposed to introduce propranolol at low dose (80 mg per day) with a great effect. The cavernoma was neither removed nor irradiated. We report the case of a patient presenting an unilateral tremor revealing a left hemispheric cavernoma. Cavernoma may be responsible for a wide variety of movement disorders depending on their location within the central nervous system. Midbrain cavernoma can cause Holmes tremor by interrupting the cerebello-rubrothalamic pathway and/or palatal myoclonus following degeneration of the inferior olivary nucleus due to a primary lesion in the dento-rubro-olivary pathway [2]. Furthermore, Südmeyer et al. [3] have reported the case of a patient with a brainstem cavernoma who presented with an asymmetric action tremor mimicking ET. Finally, cavernoma of the basal ganglia may also be responsible for movement disorders such as hemi-chorea when located in the caudate nuclei, segmental hémidystonia and hemi-parkinsonism. Here, we present the case of a patient presenting with unilateral tremor revealing a left hemispheric cavernoma. We hypothesized that the cavernoma disrupts fibers linking motor areas to the thalamus, altering neural communication within this motor network, http://dx.doi.org/10.1016/j.clineuro.2014.07.033 0303-8467/© 2014 Elsevier B.V. All rights reserved.
Fig. 1. Brain MRI showing (a) a left rounded hypointensity on T2 echo gradient sequences in the posterior cingulate gyrus and (b) its view in sagital T1 sequences.
thus leading to the controlateral tremor. In fact, electrophysiological studies on ET have demonstrated that pre-motor cortex, primary motor cortex and thalamus are parts of the oscillating network involved in ET and are closely linked to the cerebellum and the brainstem [4]. Finally, we cannot exclude that tremor in our patient can be essential in nature and that the cavernoma is an incidental finding. In conclusion, our observation emphasizes that brain imaging is needed to explore tremor mimicking asymmetric essential tremor, particularly for young adults. Conflict of interest The authors declare no conflicts of interest. Authors contributions Romain Lefaucheur and David Maltête: acquisition of data, analysis and interpretation, critical revision of the manuscript for important intellectual content, study supervision. Stéphane Derrey, Axel Lebas, David Wallon: acquisition of data, analysis and interpretation. Acknowledgment We thank Dr A. Lebas for his help to create brain MRI figure with his software JimboDicomviewer. Appendix A. Supplementary data Supplementary material related to this article can be found, in the online version, at http://dx.doi.org/10.1016/j.clineuro. 2014.07.033.
238
Letter to the Editor / Clinical Neurology and Neurosurgery 125 (2014) 237–238
References [1] Zabramski JM, Wascher TM, Spetzler RF, Johnson B, Golfinos J, Drayer BP, et al. The natural history of familial cavernous malformations: results of an ongoing study. J Neurosurg 1994;80(3):422–32. [2] Maduri R, Barbagallo G, Iofrida G, Signorelli M, Signorellli F. Regression of Benedikt’s syndrome after single-stage removal of mesencephalic cavernoma and temporal meningioma: a case report. Clin Neurol Neurosurg 2013;115(6):748–50. [3] Südmeyer M, Maroof P, Saleh A, Hartmann C, Wojtecki L, Schnitzler A. Action tremor caused by olivary cavernoma in Klippel–Trénaunay syndrome mimicking asymmetric essential tremor. J Neurol 2011;258(1):140–2. [4] Schnitzler A, Münks C, Butz M, Timmermann L, Gross J. Synchronized brain network associated with essential tremor as revealed by magnetoencephalography. Mov Disord 2009;24(11):1629–35.
Romain Lefaucheur ∗ Department of Neurology, Rouen University Hospital and University of Rouen, Rouen Cedex, France Stéphane Derrey Department of Neurosurgery, Rouen University Hospital and University of Rouen, Rouen Cedex, France Axel Lebas Department of Neurophysiology, Rouen University Hospital and University of Rouen, Rouen Cedex, France
David Wallon Department of Neurology, Rouen University Hospital and University of Rouen, Rouen Cedex, France a
David Maltête a,b Department of Neurology, Rouen University Hospital and University of Rouen, Rouen Cedex, France b INSERM U614, Rouen Faculty of Medicine, Rouen Cedex, France ∗ Corresponding
author at: Department of Neurology, Rouen University Hospital, 76031 Rouen Cedex, France. Tel.: +33 2 32 88 87 40; fax: +33 2 32 88 87 41. E-mail address: [email protected] (R. Lefaucheur) 8 May 2014 Available online 7 August 2014