Parkinsonism and Related Disorders 20 (2014) 673e675
Contents lists available at ScienceDirect
Parkinsonism and Related Disorders journal homepage: www.elsevier.com/locate/parkreldis
Letter to the Editor
Holmes’ and palatal tremor following brainstem haemorrhage Keywords: Holmes’ tremor Palatal tremor Hypertrophic olivary degeneration Brainstem haemorrhage
Holmes’ tremor is a rare symptomatic movement disorder characterised by resting tremor, enhanced by posture and further exacerbated by kinetic action. Palatal tremor is a distinct movement disorder characterised by rhythmic palatal contractions. Hypertrophic olivary degeneration (HOD) is a rare trans-synaptic degeneration that results from injury to the GuillaineMollaret triangle [1]. Co-existence of Holmes’ and palatal tremor is rare. We herein present a patient who developed a combination of Holmes’ and palatal tremor in association with HOD, 6 months following brainstem haemorrhage. A 51-year-old man presented with a continuous left upper extremity tremor, present at rest and exacerbated by activity. The tremor developed approximately 6 months after a brainstem haemorrhagic stroke (Fig. 1AeC). The tremor did not occur during sleep. Clinical examination demonstrated a coarse, irregular, predominantly proximal, low frequency resting tremor of the left upper limb that was amplified by posture and further accentuated by goal directed movements. A ‘wing beating’ component was manifest with the arms flexed horizontally (video 1, segments 1e 3). Palatal tremor was also noted with associated subtle pharyngeal movements (video 1, segment 4). There was no tremor of the head, larynx, diaphragm or other extremities. He had square wave jerks and gaze evoked nystagmus which was asynchronous with the palatal tremor, right peripheral facial palsy and left sided hemiparesis. T2-weighted magnetic resonance imaging (MRI) of the brain demonstrated hyperintense and hypertrophied lesion in the right inferior olivary nucleus consistent with hypertrophic olivary degeneration (Fig. 1D). Supplementary video related to this article can be found online at http://dx.doi.org/10.1016/j.parkreldis.2014.03.005. HOD is an unusual transynaptic degeneration secondary to injury to the GuillaineMollaret triangle, composed of the contralateral dentate nucleus, the ipsilateral red and inferior olivary nucleus (dentatoerubroeolivary pathway) [1,2]. A unique feature of this form of transneuronal degeneration is enlargement rather than atrophy of the affected structure [2]. The lesion causing HOD is usually in the ipsilateral brainstem or contralateral dentate nucleus [2,3]. Common brainstem insults leading to HOD include haemorrhage, infarction, demyelination, tumours and infections [2e6]. Brain MRI may show non-enhancing T1 isointense and T2 hyperintense enlargement of the affected olive. The evolution of http://dx.doi.org/10.1016/j.parkreldis.2014.03.005 1353-8020/Ó 2014 Elsevier Ltd. All rights reserved.
HOD is radiologically well characterized [2]. Hyperintense olivary signal on T2 weighted MRI usually develops by 1 month after the inciting lesion and may persist for a number of years or indefinitely; olivary hypertrophy develops approximately 6 months after the event and usually persists for 3e4 years, ultimately leading to shrinkage [2,5]. Palatal tremor is the usual signature clinical syndrome of HOD but Holmes’ tremor may also occur [2e6]. Coexistence is uncommon and autopsy findings suggest that the extent of involvement of olive and dentate nucleus may dictate the clinical presentation [7]. Palatal tremor is characterised by rhythmic continuous movements of the soft palate and sometimes other muscles innervated by cranial or spinal nerves [4,5]. The condition may be essential or symptomatic [4]. Patients with symptomatic palatal tremor (SPT) may manifest other clinical features of brainstem or cerebellar dysfunction. Development of SPT is temporally associated with signal changes and hypertrophy of the olivary region [2,5]. In most cases, SPT may appear within few weeks to a few months after an insult to the GuillaineMollaret triangle, reaching a peak between 5 and 24 months [5]. The Holmes’ tremor, also known as rubral or midbrain tremor is characterised by slow frequency resting tremor (usually less than 4.5 Hz), enhanced by posture and further exacerbated by intentional movements [4]. The traditional terms rubral and midbrain tremor can be misleading as the red nucleus and midbrain are not the sole anatomical substrates in the disorder [4]. A variety of insults to the brainstem, cerebellum and thalamus can lead to this symptomatic tremor [3,4,6]. Holmes’ tremor usually arises following a delay of 4 weeks to 2 years following an acute brainstem insult and does not necessarily correlate with HOD [3,4,6]. Structural and functional neuroimaging studies indicate that the disorder may result from disruption of the cerebellorubrothalamic and nigrostriatal pathways [6]. Our case demonstrates the rare co-occurrence of Holmes’ and palatal tremor in association with HOD. Although the temporal evolution of the two clinical syndromes could not be established in our case, the clinico-radiological profile illustrates the role of dentatoe rubroeolivary circuitry in the generation of two distinct interesting tremor syndromes. Author contributions Suresh Kumar Chhetri: study concept and design, drafting/ revising the manuscript for content, data interpretation, coordination and execution of the study. Sachin Mathur; Mian Ayaz Ul Haq: acquisition of data, review and critique of the manuscript. Bernard Boothman; Tahir Majeed: acquisition of data, revising the manuscript for intellectual content and supervision.
674
Letter to the Editor / Parkinsonism and Related Disorders 20 (2014) 673e675
Fig. 1. CT scan showing haemorrhage in the right side of midbrain which extended to the pontomedullary junction (A). MRI T2 axial image performed two weeks after the ictus showing high signal consistent with haemorrhage in the pons and extending posteriorly to the right superior cerebellar peduncle (B). Coronal pre-contrast T1 sequence showing haemorrhage in the ponto-medullary junction. This is located in the region of Guillain-Mollaret triangle (C). MRI T2 axial image, 6 months after haemorrhage showing hyperintensity and hypertrophy of the right inferior olivary nucleus in keeping with hypertrophic olivary degeneration (D).
Study funding No targeted funding reported. Disclosure Dr. Chhetri has received educational grant from the George Barton Motor Neurone Disease Trust.
Acknowledgements The authors thank Catherine Lamoon, senior medical photographer, Royal Preston Hospital for her help with the videos.
References [1] Guillain G, Mollaret P. Deux cas de myoclonies synchrones et rhythmes velopharyngo-laryngo-oculo-diaphragmatiques. Rev Neurol 1931;2:545e66. [2] Goyal M, Versnick E, Tuite P, Cyr JS, Kucharczyk W, Montanera W, et al. Hypertrophic olivary degeneration: metaanalysis of the temporal evolution of MR findings. AJNR Am J Neuroradiol 2000;21:1073e7.
[3] Rieder CR, Reboucas RG, Ferreira MP. Holmes tremor in association with bilateral hypertrophic olivary degeneration and palatal tremor: chronological considerations. Case report. Arq Neuropsiquiatr 2003;61:473e7. [4] Deuschl G, Bain P, Brin M. Consensus statement of the Movement Disorder Society on Tremor. Ad Hoc Scientific Committee. Mov Disord 1998;13(Suppl. 3):2e23. [5] Nishie M, Yoshida Y, Hirata Y, Matsunaga M. Generation of symptomatic palatal tremor is not correlated with inferior olivary hypertrophy. Brain 2002;125: 1348e57. [6] Paviour DC, Jager HR, Wilkinson L, Jahanshahi M, Lees AJ. Holmes tremor: application of modern neuroimaging techniques. Mov Disord 2006;21:2260e2. [7] Masucci EF, Kurtzke JF, Saini N. Myorhythmia: a widespread movement disorder. Clinicopathological correlations. Brain 1984;107:53e79.
Suresh Kumar Chhetri* The University of Manchester, Manchester Academic Health Science Centre, Manchester M13 9PL, United Kingdom Department of Neurology, Royal Preston Hospital, Lancashire Teaching Hospitals NHS Foundation Trust, Preston PR2 9HT, United Kingdom Bernard Boothman, Mian Ayaz Ul Haq Department of Neurology, Royal Preston Hospital, Lancashire Teaching Hospitals NHS Foundation Trust, Preston PR2 9HT, United Kingdom
Letter to the Editor / Parkinsonism and Related Disorders 20 (2014) 673e675
Sachin Mathur Department of Neuroradiology, Royal Preston Hospital, Lancashire Teaching Hospitals NHS Foundation Trust, Preston PR2 9HT, United Kingdom Tahir Majeed Department of Neurology, Royal Preston Hospital, Lancashire Teaching Hospitals NHS Foundation Trust, Preston PR2 9HT, United Kingdom
675
* Corresponding
author. Department of Neurology, Royal Preston Hospital, Lancashire Teaching Hospitals NHS Foundation Trust, Preston PR2 9HT, United Kingdom. Tel.: þ44 1772522256; fax: þ44 1772523165. E-mail address: [email protected] (S.K. Chhetri). 25 December 2013
Contents lists available at ScienceDirect
Parkinsonism and Related Disorders journal homepage: www.elsevier.com/locate/parkreldis
Letter to the Editor
Holmes’ and palatal tremor following brainstem haemorrhage Keywords: Holmes’ tremor Palatal tremor Hypertrophic olivary degeneration Brainstem haemorrhage
Holmes’ tremor is a rare symptomatic movement disorder characterised by resting tremor, enhanced by posture and further exacerbated by kinetic action. Palatal tremor is a distinct movement disorder characterised by rhythmic palatal contractions. Hypertrophic olivary degeneration (HOD) is a rare trans-synaptic degeneration that results from injury to the GuillaineMollaret triangle [1]. Co-existence of Holmes’ and palatal tremor is rare. We herein present a patient who developed a combination of Holmes’ and palatal tremor in association with HOD, 6 months following brainstem haemorrhage. A 51-year-old man presented with a continuous left upper extremity tremor, present at rest and exacerbated by activity. The tremor developed approximately 6 months after a brainstem haemorrhagic stroke (Fig. 1AeC). The tremor did not occur during sleep. Clinical examination demonstrated a coarse, irregular, predominantly proximal, low frequency resting tremor of the left upper limb that was amplified by posture and further accentuated by goal directed movements. A ‘wing beating’ component was manifest with the arms flexed horizontally (video 1, segments 1e 3). Palatal tremor was also noted with associated subtle pharyngeal movements (video 1, segment 4). There was no tremor of the head, larynx, diaphragm or other extremities. He had square wave jerks and gaze evoked nystagmus which was asynchronous with the palatal tremor, right peripheral facial palsy and left sided hemiparesis. T2-weighted magnetic resonance imaging (MRI) of the brain demonstrated hyperintense and hypertrophied lesion in the right inferior olivary nucleus consistent with hypertrophic olivary degeneration (Fig. 1D). Supplementary video related to this article can be found online at http://dx.doi.org/10.1016/j.parkreldis.2014.03.005. HOD is an unusual transynaptic degeneration secondary to injury to the GuillaineMollaret triangle, composed of the contralateral dentate nucleus, the ipsilateral red and inferior olivary nucleus (dentatoerubroeolivary pathway) [1,2]. A unique feature of this form of transneuronal degeneration is enlargement rather than atrophy of the affected structure [2]. The lesion causing HOD is usually in the ipsilateral brainstem or contralateral dentate nucleus [2,3]. Common brainstem insults leading to HOD include haemorrhage, infarction, demyelination, tumours and infections [2e6]. Brain MRI may show non-enhancing T1 isointense and T2 hyperintense enlargement of the affected olive. The evolution of http://dx.doi.org/10.1016/j.parkreldis.2014.03.005 1353-8020/Ó 2014 Elsevier Ltd. All rights reserved.
HOD is radiologically well characterized [2]. Hyperintense olivary signal on T2 weighted MRI usually develops by 1 month after the inciting lesion and may persist for a number of years or indefinitely; olivary hypertrophy develops approximately 6 months after the event and usually persists for 3e4 years, ultimately leading to shrinkage [2,5]. Palatal tremor is the usual signature clinical syndrome of HOD but Holmes’ tremor may also occur [2e6]. Coexistence is uncommon and autopsy findings suggest that the extent of involvement of olive and dentate nucleus may dictate the clinical presentation [7]. Palatal tremor is characterised by rhythmic continuous movements of the soft palate and sometimes other muscles innervated by cranial or spinal nerves [4,5]. The condition may be essential or symptomatic [4]. Patients with symptomatic palatal tremor (SPT) may manifest other clinical features of brainstem or cerebellar dysfunction. Development of SPT is temporally associated with signal changes and hypertrophy of the olivary region [2,5]. In most cases, SPT may appear within few weeks to a few months after an insult to the GuillaineMollaret triangle, reaching a peak between 5 and 24 months [5]. The Holmes’ tremor, also known as rubral or midbrain tremor is characterised by slow frequency resting tremor (usually less than 4.5 Hz), enhanced by posture and further exacerbated by intentional movements [4]. The traditional terms rubral and midbrain tremor can be misleading as the red nucleus and midbrain are not the sole anatomical substrates in the disorder [4]. A variety of insults to the brainstem, cerebellum and thalamus can lead to this symptomatic tremor [3,4,6]. Holmes’ tremor usually arises following a delay of 4 weeks to 2 years following an acute brainstem insult and does not necessarily correlate with HOD [3,4,6]. Structural and functional neuroimaging studies indicate that the disorder may result from disruption of the cerebellorubrothalamic and nigrostriatal pathways [6]. Our case demonstrates the rare co-occurrence of Holmes’ and palatal tremor in association with HOD. Although the temporal evolution of the two clinical syndromes could not be established in our case, the clinico-radiological profile illustrates the role of dentatoe rubroeolivary circuitry in the generation of two distinct interesting tremor syndromes. Author contributions Suresh Kumar Chhetri: study concept and design, drafting/ revising the manuscript for content, data interpretation, coordination and execution of the study. Sachin Mathur; Mian Ayaz Ul Haq: acquisition of data, review and critique of the manuscript. Bernard Boothman; Tahir Majeed: acquisition of data, revising the manuscript for intellectual content and supervision.
674
Letter to the Editor / Parkinsonism and Related Disorders 20 (2014) 673e675
Fig. 1. CT scan showing haemorrhage in the right side of midbrain which extended to the pontomedullary junction (A). MRI T2 axial image performed two weeks after the ictus showing high signal consistent with haemorrhage in the pons and extending posteriorly to the right superior cerebellar peduncle (B). Coronal pre-contrast T1 sequence showing haemorrhage in the ponto-medullary junction. This is located in the region of Guillain-Mollaret triangle (C). MRI T2 axial image, 6 months after haemorrhage showing hyperintensity and hypertrophy of the right inferior olivary nucleus in keeping with hypertrophic olivary degeneration (D).
Study funding No targeted funding reported. Disclosure Dr. Chhetri has received educational grant from the George Barton Motor Neurone Disease Trust.
Acknowledgements The authors thank Catherine Lamoon, senior medical photographer, Royal Preston Hospital for her help with the videos.
References [1] Guillain G, Mollaret P. Deux cas de myoclonies synchrones et rhythmes velopharyngo-laryngo-oculo-diaphragmatiques. Rev Neurol 1931;2:545e66. [2] Goyal M, Versnick E, Tuite P, Cyr JS, Kucharczyk W, Montanera W, et al. Hypertrophic olivary degeneration: metaanalysis of the temporal evolution of MR findings. AJNR Am J Neuroradiol 2000;21:1073e7.
[3] Rieder CR, Reboucas RG, Ferreira MP. Holmes tremor in association with bilateral hypertrophic olivary degeneration and palatal tremor: chronological considerations. Case report. Arq Neuropsiquiatr 2003;61:473e7. [4] Deuschl G, Bain P, Brin M. Consensus statement of the Movement Disorder Society on Tremor. Ad Hoc Scientific Committee. Mov Disord 1998;13(Suppl. 3):2e23. [5] Nishie M, Yoshida Y, Hirata Y, Matsunaga M. Generation of symptomatic palatal tremor is not correlated with inferior olivary hypertrophy. Brain 2002;125: 1348e57. [6] Paviour DC, Jager HR, Wilkinson L, Jahanshahi M, Lees AJ. Holmes tremor: application of modern neuroimaging techniques. Mov Disord 2006;21:2260e2. [7] Masucci EF, Kurtzke JF, Saini N. Myorhythmia: a widespread movement disorder. Clinicopathological correlations. Brain 1984;107:53e79.
Suresh Kumar Chhetri* The University of Manchester, Manchester Academic Health Science Centre, Manchester M13 9PL, United Kingdom Department of Neurology, Royal Preston Hospital, Lancashire Teaching Hospitals NHS Foundation Trust, Preston PR2 9HT, United Kingdom Bernard Boothman, Mian Ayaz Ul Haq Department of Neurology, Royal Preston Hospital, Lancashire Teaching Hospitals NHS Foundation Trust, Preston PR2 9HT, United Kingdom
Letter to the Editor / Parkinsonism and Related Disorders 20 (2014) 673e675
Sachin Mathur Department of Neuroradiology, Royal Preston Hospital, Lancashire Teaching Hospitals NHS Foundation Trust, Preston PR2 9HT, United Kingdom Tahir Majeed Department of Neurology, Royal Preston Hospital, Lancashire Teaching Hospitals NHS Foundation Trust, Preston PR2 9HT, United Kingdom
675
* Corresponding
author. Department of Neurology, Royal Preston Hospital, Lancashire Teaching Hospitals NHS Foundation Trust, Preston PR2 9HT, United Kingdom. Tel.: þ44 1772522256; fax: þ44 1772523165. E-mail address: [email protected] (S.K. Chhetri). 25 December 2013
