A 40-year-old man presented with a 6-month history of mild but worsening tremor of variable intensity. The patient had a focal, irregular, rest and action tremor of middle amplitude and low frequency (about 4 Hz), limited to his right arm with oscillatory motion around the elbow. It was present inconsistently at rest, particularly during emotional activation, and enhanced by posture maintenance. It was evoked by various positions and tasks. Particularly, the tremor was present during fine motor skills such as writing, and it was increased by drinking and shaving, showing a minimal intentional component. The patient also reported slight loss in manual dexterity in his right hand

Carlos, Arenn Faye

Ceroni, Mauro

Davin, Annalisa

Galli, Alberto

Guaita, Antonio

Medici, Valentina

Poloni, Tino Emanuele

Riva, Emanuela

English

Poloni T. E.

Galli A.

Carlos A. F.

Riva E.

Medici V.

Davin A.

Guaita A.

Ceroni M.

Archivio Istituzionale della Ricerca - Università degli Studi di Pavia

Reversible Holmes Tremor due to Middle Cerebral Artery Giant Aneurysm

Columbia University Academic Commons

1New Observations LettersReversible Holmes Tremor due to Middle Cerebral Artery Giant AneurysmTino Emanuele Poloni1,2*, Alberto Galli3, Arenn Faye Carlos1, Emanuela Riva2,  Valentina Medici1, Annalisa Davin1, Antonio Guaita1 & Mauro Ceroni1,41Department of Neurology and Neuropathology, Golgi-Cenci Foundation, Milan, IT, 2Department of Rehabilitation, ASP Golgi-Redaelli, Milan, IT, 3Department of Neurology, San Carlo Borromeo Hospital, Milan, IT, 4Department of Neurological Science, IRCCS “Istituto Neurologico C. Mondino” Foundation, University of Pavia, Pavia, ITIn 1904, Gordon Holmes first described a peculiar tremor that now bears his name. The tremor Holmes depicted was present at rest (except during complete relaxation) and was exacerbated by both voluntarily maintaining postures and movements. Holmes tremor (HT) is a symptom-atic, mostly unilateral, low-frequency (<5 Hz) tremor. Depending on the extent of  the underlying lesion, HT is usually associated with other neu-rological manifestations such as sensorimotor deficits, bradykinesia, mus-cle tone alterations, ophthalmoparesis, and ataxia.1 Several thalamic or midbrain lesions may produce HT including stroke,2 arteriovenous mal-formations,3 traumatic injuries,4 inflammatory or infective diseases,5 and tumors.2 Although previously known as “rubral tremor,” HT is the pre-ferred nomenclature nowadays by experts considering that single lesions of  the red nucleus are insufficient to generate persistent tremor. Indeed, the generation of  HT requires a dysfunction of  both dopaminergic and non-dopaminergic circuitries. In particular, the resting and postural com-ponents may be triggered by nigrostriatal damage,6 while the action com-ponents may be due to the involvement of  dentato-rubro and cerebello-thalamic tracts.3,7 Typically, HT develops months after the lesion, probably requiring concomitant degeneration and aberrant regen-eration of  involved circuits. HT pathogenesis is complex and different in each case. Consequently, the functional neuroimaging of  basal ganglia Dopamine Transporter scan (DaT-scan), Single-Photon Emission Computed Tomography (SPECT), Positron Emission Tomography (PET) gives inconsistent results,6,8 and the response to symptomatic drugs, l-dopa, and Deep Brain Stimulation (DBS) of  different targets such as Ventral Intermediate Nucleus (VIM), SubThalamic Nucleus (STN), Globus Pallidus Internus (GPI), prelemniscal radiations is variable and almost unpredictable.2,9 Almost all recounted cases have complex clinical syndromes, and a complete clinical recovery has never been reported.A 40-year-old man presented with a 6-month history of  mild but wors-ening tremor of  variable intensity. The patient had a focal, irregular, rest and action tremor of  middle amplitude and low frequency (about 4 Hz), limited to his right arm with oscillatory motion around the elbow. It was present inconsistently at rest, particularly during emotional activation, and enhanced by posture maintenance. It was evoked by various positions and tasks. Particularly, the tremor was present during fine motor skills such as writing, and it was increased by drinking and shaving, showing a minimal intentional component. The patient also reported slight loss in manual dexterity in his right hand. A minimal hypokinesia was present in the patient’s right arm (Video 1). The neurological examination was oth-erwise normal and did not show bradykinesia or muscle tone alteration. There was no history of  neurodegenerative disease in his family. Columbia University LibrariesFreely available onlineTremor and Other Hyperkinetic Movementshttp://www.tremorjournal.orgAbstractKeywords: Hemiparkinsonism, Holmes tremor, giant aneurysm, meso-diencephalic lesions, rubral tremor, tremor differential diagnosis, tremor surgical treatmentCitation: Poloni TE, Galli A, Carlos AF, Riva E, Medici V, Davin A, et al. Reversible Holmes Tremor Due to Middle Cerebral Artery Giant Aneurysm. Tremor Other Hyperkinet Mov. 2019; 9. doi: 10.7916/tohm.v0.695.*To whom correspondence should be addressed. E-mail: tepoloni@gmail.com or e.poloni@golgicenci.itEditor: Elan D. Louis, Yale University, USAReceived: June 27, 2019; Accepted: August 5, 2019; Published: September 4, 2019Copyright: © 2019 Poloni et al. This is an open-access article distributed under the terms of  the Creative Commons Attribution–Noncommercial–No Derivatives License, which permits the user to copy, distribute, and transmit the work provided that the original authors and source are credited; that no commercial use is made of  the work; and that the work is not altered or transformed.Funding: None.Financial Disclosures: None.Conflicts of Interest: The authors report no conflicts of  interest.Ethics Statement: All patients who appear on the video have provided written informed consent; authorization for the videotaping and the publication of  the videotape was obtained.Poloni TE, Galli A, Carlos AF, et al. Holmes Tremor Due to Giant AneurysmColumbia University LibrariesTremor and Other Hyperkinetic Movementshttp://www.tremorjournal.org 2Nevertheless, two of  his first-degree relatives had previously died from a ruptured cerebral aneurysm. The patient underwent brain magnetic resonance imaging (MRI) that revealed a space-occupying lesion resem-bling an onion bulb. The lesion, a giant left middle cerebral artery bifurcation aneurysm with almost complete thrombosis, was compress-ing the left diencephalon, involving the thalamus, basal ganglia, and left upper mesencephalon at the level of  substantia nigra and red nucleus (Figure 1). Due to the clear etiology of  the tremor, the DaT-scan imag-ing was avoided and the patient underwent surgery. The total excision of  the aneurysm led to an almost complete disappearance of  the tremor at the first neurological check, 1 month after surgery. The patient was followed clinically every year, and at the last follow-up (12 years after surgery), the patient maintained a virtually normal neurological exam-ination. Currently, the tremor is no longer detectable, except for a min-imal postural tremor manifesting in his right arm only during isometric effort and at certain positions (Video 1). The patient also describes a minimal subjective loss of  movement  fluidity in his right hand.Although mild, isolated, and primarily action related, the herein reported tremor shows the main characteristics of  HT according to the current consensus statement on the classification of  tremors.10 Indeed, our patient has a rest, postural, and intention tremor of  the right arm at low frequency (<5 Hz). Moreover, the tremor resulted secondary to an acquired lesion in the vicinity of  the red nucleus. This unique and mild HT case caused by a rare lesion widens the spectrum of  conditions causing HT. Indeed, giant aneurysms have never been reported as a possible cause of  HT. Gross and Sibon had reported two similar cases of  middle cerebral artery giant aneurysm causing “hemiparkinsonism” characterized by lateralized pyramidal and extrapyramidal signs and tremor.11,12 Bostantjopoulou also described a case of  hemiparkinsonism due to a similar aneurysm, where the tremor was predominantly present at rest.13 In these cases, the tremor was part of  an obvious parkinsonian syndrome, which lacks in our case. Moreover, HT usually occurs with other localizing signs, and it has always been reported as part of  severe or complex motor syndromes. Compared with other recounted cases of  HT, our case showed an almost isolated and focal tremor syndrome. Thus, we emphasize that HT may also present as a pure, focal, rest and action tremor suggesting a meso-diencephalic lesion rather than an incipi-ent Parkinson’s disease or an essential tremor. Therefore, it is crucial to recognize this syndrome and look for a possible lesion in the afore-mentioned anatomical region. Additionally, our HT case is relatively mild and reversible due to the gradually compressive and non- destructive nature of  the lesion; the very slow growth of  the aneu-rysm probably only caused a dysfunction of  the nigro-striatal and dentato-rubro-thalamic pathways. In spite of  a concomitant com-pression of  the left cerebral peduncle, pyramidal signs were minimal consisting of  a slight loss of  manual dexterity in patient’s right hand. The pyramidal tract was probably dislocated but not damaged by the aneurysm. The patient was cured by surgery: we emphasize the reversible nature of  HT in peculiar cases.Figure 1.  Patient’s MRI and graphic test. (A–C) MRI shows the giant aneurysm compressing substantia nigra, red nucleus, basal ganglia, and thalamus. (C) Gadolinium enhancement of  the residual open component of  the aneurysm. (D) Complete removal of  the aneurysm. (E and F) Patient’s graphic tests before and after surgery.Poloni TE, Galli A, Carlos AF, et al. Holmes Tremor Due to Giant AneurysmColumbia University LibrariesTremor and Other Hyperkinetic Movementshttp://www.tremorjournal.org 3AcknowledgmentsThe authors would like to thank the student Giulia Bortone for her precious technical support.References1. Holmes G. On certain tremors in organic cerebral lesions. Brain 1904;27:327–375. doi: 10.1093/brain/27.3.3272. Raina GB, Cerosimo MG, Folgar SS, et al. Holmes tremor clinical descrip-tion, lesion localization, and treatment in a series of  29 cases. Neurology 2016;86:931–938. doi: 10.1212/WNL.00000000000024403. Menéndez DFS, Cury RG, Barbosa ER, Teixeira MJ, Fonoff  E. Hypertrophic olivary degeneration and Holmes’ tremor secondary to bleeding of  cavernous malformation in the midbrain. Tremor Other Hyperkinet Mov 2014;4:264. doi: 10.7916/D8PG1PXT4. Cury RG, Barbosa ER, Freitas C, de Souza Godoy LF, Paiva WS. Holmes tremor secondary to a stabbing lesion in the midbrain. Tremor Other Hyperkinet Mov 2017;7:523. doi: 10.7916/D8TF08ZT5. Katshnig-Winter P, Koegl-Wallner M, Pendl T, Fazekas F, Schwingenschuh P. Levodopa-responsive Holmes’ tremor caused by a single inflammatory demyelinating lesion. Tremor Other Hyperkinet Mov 2015;5:339. doi: 10.7916/D8WQ033X6. Remy P, de Recondo A, Defer G, et al. Peduncular “rubral” tremor and dopaminergic denervation: a PET study. Neurology 1995;45:472–477. doi: 10.1212/WNL.45.3.4727. Vidailhet M, Jedynak CP, Pollak P, Agid Y. Pathology of  symptomatic trem-ors. Mov Disord 1998;13:49–54. doi: 10.1002/mds.8701313098. Gajos A, Budrewiez S, Kosszewiez M, et al. Is nigrostratal dopaminergic defi-cit necessary for Holmes tremor to develop? The DaTSCAN and IBZM SPECT study. J Neural Transm 2017;124:1389–1393. doi: 10.1007/s00702-017-1780-19. Martinez V, Hu SC, Foutz TJ, Ko A. Successful treatment of  Holmes tremor with deep brain simulation of  the prelemniscal radiations. Front Surg 2018;5:21. doi: 10.3389/fsurg.2018.0002110. Bhatia KP, Bain P, Bajaj N, et al. Consensus statement on the classification of  tremors. From the task force on tremor of  the International Parkinson and Movement Disorder Society. Mov Disord 2018;33:75–87. doi: 10.1002/mds.2712111. Gross M. Giant middle cerebral aneurysm presenting as hemiparkinsonism. J Neurol Neurosurg Psychiatry 1987;50:1075. doi: 10.1136/jnnp.50.8.107512. Sibon I, Rajabally Y, Tison F. Parkinsonism as a result of  a giant  aneurysm. Mov Disord 1999;14:159–161. doi: 10.1002/ 1531-8257 (199901) 14:1<159::AID-MDS1029>3.3.CO;2-D13. Bostantjopoulou S, Katsarou Z, Petridis A, Andreou A. Aneurysm present-ing as parkinsonism. Neurology 2006;67:2028. doi: 10.1212/01.wnl.0000232726. 76501.09Video 1. Tremor Clinical Features: This Video Shows the Clinical Manifestations of  the Tremor Prior to the Surgical Excision of  the Aneurysm Compared to the Clinical Picture Following the Surgery and Lasting up to 12 Years (Last Follow-up). In sequence, it shows postural tremor, postural tremor during isometric effort (handgrip), movement fluidity in pronation/supination test, and kinetic tremor during fine motor skills (graphic tests).

https://academiccommons.columbia.edu/doi/10.7916/d8-sgk8-sp37/download

Reversible Holmes Tremor due to Middle Cerebral Artery Giant Aneurysm

Abstract

Similar works

Full text

Available Versions

Archivio Istituzionale della Ricerca - Università degli Studi di Pavia

Columbia University Academic Commons