Background: The dentatothalamic tract connects the dentate nucleus of the cerebellum with the contralateral thalamus and plays a major role in the pathogenesis of tremor. Unilateral lesions of the dentatothalamic pathway may affect its ipsilateral predecussational or its contralateral postdecussational course, which results either in ipsilateral or in contralateral tremor.

Case Report: Here, we present two patients with a unilateral midbrain lesion resulting in bilateral tremor. Both patients presented with severe kinetic tremor.

Discussion: The corresponding unilateral mesencephalic lesion affected both the ipsilateral predecussational and the ipsilateral postdecussational dentatothalamic tract originating from the contralateral dentate nucleus, which is very unusual and has not been outlined clearly before

Esmaeilzadeh, Majid

Krauss, Joachim K.

Uksul, Nesrin

Tremor and Other Hyperkinetic Movements

English

Columbia University Academic Commons

Case ReportsSevere Bilateral Kinetic Tremor Due to Unilateral Midbrain LesionsMajid Esmaeilzadeh1*, Nesrin Uksul1& Joachim K. Krauss11Department of Neurosurgery, Hannover Medical School, Hannover, GermanyAbstractBackground: The dentatothalamic tract connects the dentate nucleus of the cerebellum with the contralateral thalamus and plays a major role in the pathogenesisof tremor. Unilateral lesions of the dentatothalamic pathway may affect its ipsilateral predecussational or its contralateral postdecussational course, which resultseither in ipsilateral or in contralateral tremor.Case Report: Here, we present two patients with a unilateral midbrain lesion resulting in bilateral tremor. Both patients presented with severe kinetic tremor.Discussion: The corresponding unilateral mesencephalic lesion affected both the ipsilateral predecussational and the ipsilateral postdecussational dentatothalamictract originating from the contralateral dentate nucleus, which is very unusual and has not been outlined clearly before.Keywords: Dentatothalamic tract, midbrain, superior cerebellar peduncle, tremor, brainstemCitation: Esmaeilzadeh M, Uksul N, Krauss JK. Severe bilateral kinetic tremor due to unilateral midbrain lesions. Tremor Other Hyperkinet Mov. 2017; 7.doi: 10.7916/D88G8Z8J* To whom correspondence should be addressed. E-mail: esmaeilzadeh.majid@mh-hannover.deEditor: Elan D. Louis, Yale University, USAReceived: September 30, 2017 Accepted: November 10, 2017 Published: December 6, 2017Copyright: ’ 2017 Esmaeilzadeh et al. This is an open-access article distributed under the terms of the Creative Commons Attribution–Noncommercial–No Derivatives License, whichpermits 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 notaltered or transformed.Funding: None.Financial Disclosures: None.Conflicts of Interest: The authors report no conflict of interest.Ethics Statement: This study was performed in accordance with the ethical standards detailed in the Declaration of Helsinki. For this type of study, formal approval of the institutionalethics committee is not required at the authors’ institution.IntroductionMidbrain lesions may cause a variety of different tremors dependingon the exact site of the lesion within the midbrain.1–4 Patients withunilateral tremor may harbor an ipsilateral or a contralateral lesionaccording to its location along the dentatothalamic pathway, but inpatients with bilateral tremor usually bilateral lesions are identified.3,5,6It is exceedingly uncommon, however, that a unilateral lesion may causebilateral tremor.Case reportsPatient 1A 55-year-old female was admitted after she lost consciousness.Magnetic resonance imaging (MRI) demonstrated a right-sided mesen-cephalic hemorrhage with intraventricular bleeding. A ventricular drainwas placed for cerebrospinal fluid diversion. The patient recoveredslowly over the next few weeks. Four weeks after the hemorrhage shedeveloped severe ataxia and postural instability. In addition, bilateralkinetic tremor of the upper extremities became evident (frequency 5 Hz,variable amplitude up to 8 cm). The tremor was scored according tothe Fahn–Tolosa–Marin Tremor Scale: at rest 5 0, with posture 53 (right) and 4 (left), and with action 5 4.7,8 Repeat MRI showedpartial resorption of the hemorrhage. Digital subtraction angio-graphy showed an arteriovenous malformation (Spetzler–Martingrade III) within the tectum and tegmentum of the right mesen-cephalon extending to the superior cerebellar peduncle. The arterio-venous malformation was partially embolized and then resected viaa midline suboccipital craniotomy.Postoperatively there was no change in tremor severity. Medicationwith levodopa, gabapentin and tiapride had no effect on tremorseverity, but clonazepam yielded mild amelioration; however, it had tobe stopped because of sedation. MRI at 5 months after the hemor-rhage demonstrated a circumscribed lesion in the dorsal aspect of theright mesencephalon extending from the superior cerebellar peduncleup to the mesencephalic-diencephalic transition zone (Figure 1A,B).The patient recovered slowly, although she did not become fully inde-pendent. The tremor improved spontaneously and no further medi-cation was needed.Freely available onlineTremor and Other Hyperkinetic Movementshttp://www.tremorjournal.orgThe Center for Digital Research and ScholarshipColumbia University Libraries/Information Services1Patient 2A 41-year-old female presented with a 10-year history of slowly pro-gressive left-sided hemiparesis, dystonia of her left hand, double vision,and ataxia. Initial MRI had demonstrated a small non-enhancing mesen-cephalic tumor, while recent studies showed an enhancing brainstemtumor located at the pontomesencephalic junction on the right side(Figure 2A,B). A stereotactic biopsy was performed, and histologicalexamination revealed a glioblastoma World Health Organization grade IV.Despite combined radiochemotherapy there was a substantial increasein tumor volume. In parallel, the patient developed bilateral kinetictremor of both upper extremities (frequency 4 Hz, amplitude 5–7 cm)as well as marked ataxia and dysarthria. Ratings on the Fahn–Tolosa–Marin Tremor Scale were the following: at rest 5 0, with posture 5 3,and with action 5 4. Subtotal tumor resection was achieved by atelovelar approach via a midline suboccipal craniotomy.Postoperatively, there was a mild transient increase in the right-sided tremor. During the next 2 years the situation stabilized underchemotherapy with temozolamid and bevacizumap. Subsequently,however, there was an increase in dysarthria and ataxia, and the patientbecame bedridden. She also suffered from an increase in right-sidedhemiparesis with parallel disappearance of the right-sided tremor.MRI demonstrated massive growth of the residual tumor. The patientsuccumbed within the next few weeks.DiscussionKinetic tremors as described in our patients have variously beenlabeled as midbrain tremor, rubral tremor, cerebellar tremor, orhyperkinesia volitionnelle.4,9 When such tremors are combined witha prominent tremor at rest, they may also be categorized as Holmestremor according to a consensus statement.9The anatomical correlate for kinetic tremor most frequently is alesion of the dentatothalamic pathway along its course from thedentate nucleus to the contralateral thalamus. The dentatothalamicpathway leaves the cerebellum within the superior cerebellar peduncleentering the tectum of the midbrain and crosses to the contralateralside within the decussation of the superior cerebellar peduncles.10While a predecussational lesion of the dentatothalamic pathway maycause tremor ipsilateral to the lesion, a postdecussational lesion wouldresult in contralateral tremor.3,6There is a wide variety of disorders and lesions that may causemidbrain tremor, including multiple sclerosis, infection, stroke, trauma,tumor, cavernoma, or vascular malformation.1,4 The pathophysiology ofkinetic tremor has not been fully clarified. Typically, such tremors developwith a variable delay of days or weeks after the lesion has occurred.9,11,12Bilateral kinetic tremor is more common in patients with multiplesclerosis in whom multiple lesions can be identified in the cerebellumor the midbrain.1 A unilateral midbrain lesion may only cause bilateraltremor when it is located at a strategic point close to the decussation ofthe superior peduncles and if it has an appropriate size (Figure 3).10,13Figure 1. Axial T2-weighted Magnetic Resonance Imaging Scans ofPatient 1 (A,B). A hypointense lesion with a hyperintense rim in the dorsalaspect of the right mesencephalon extending from the superior cerebellarpeduncle to the mesencephalic–diencephalic transition zone.Figure 2. Axial T1-weighted Enhanced Magnetic Resonance Scans ofPatient 2 (A,B). A tumor located in the right pontine and mesencephalictectum and tegmentum.Figure 3. Schematic View of the Site of the Lesion. In both patientslocated at a strategic point close to the decussatio pedunculorum affectingthe ipsilateral predecussational dentatothalamic pathway and the ipsilateralpostdecussational dentatothalamic pathway originating from the contra-lateral dentate nucleus. (Modified from Figure 163, Nieuwenhuys et al.,Springer, 1991 (13)).Esmaeilzadeh M, Uksul N, Krauss JK Severe Bilateral Kinetic TremorTremor and Other Hyperkinetic Movementshttp://www.tremorjournal.orgThe Center for Digital Research and ScholarshipColumbia University Libraries/Information Services2With regard to the published literature, such lesions seem to beexceedingly rare. Since Jakob and Huhn5 identified a vascular lesionaffecting the mesencephalic tegmentum to be responsible for bilateraltremor during autopsy, this issue has received little attention.4As demonstrated in our patients the prognosis of bilateral tremorassociated with a unilateral lesion may depend both on the devel-opment of the lesion itself and also on the appearance of otherneurological symptoms. While reduction in the volume of the lesion,such as increased resorption of the hemorrhage, may result inimprovement of tremor, a marked increase in volume by tumorgrowth can also affect other systems such as the corticospinal tractand may result in reduction of tremor secondary to an increase inhemiparesis.In conclusion, unilateral midbrain lesions of the cerebellar outflowmay affect the ipsilateral predecussational and the ipsilateral post-decussational dentatothalamic tract originating from the contralateraldentate nucleus, which can result in bilateral kinetic tremor.References1. Deuschl G. Movement disorders in multiple sclerosis and their treatment.Neurodegener Dis Manag 2016;6:31–35. doi: 10.2217/nmt-2016-00532. Kinfe TM, Capelle HH, Krauss JK. Impact of surgical treatment ontremor due to posterior fossa tumors. J Neurosurg 2008;108:692–697. doi: 10.3171/JNS/2008/108/4/06923. Krauss JK, Wakhloo AK, Nobbe F, Tra¨nkle R, Mundinger F, Seeger W.Lesion of dentatothalamic pathways in severe post-traumatic tremor. Neurol Res1995;17:409–416.4. Vidailhet M, Jedynak CP, Pollak P, Agid Y. Pathology of symptomatictremors. Mov Disord 1998;13 Suppl 3:49–54. doi: 10.1002/mds.8701313095. Huhn B, Jakob H. Traumatic brain stem lesions with long-term survival.Contribution to the pathology of substancia nigra and the pontine syndrome.Nervenarzt 1970;41:326–334.6. von Cramon D. Bilateral cerebellar dysfunctions in a unilateral meso-diencephalic lesion. J Neurol Neurosurg Psychiatry 1981;44:361–363. doi: 10.1136/jnnp.44.4.3617. Fahn S, Tolosa E, Concepcion M. Clinical rating scale for tremor.In: Jankovic J, Tolosa E, editors. Parkinson’s disease and movement disorders,2nd ed. Baltimore: Williams and Wilkins; 1993. p 271–280.8. Hess CW, Pullman SL. Tremor: clinical phenomenology and assessmenttechniques. Tremor Other Hyperkinet Mov 2012;2. pii: tre-02-65-365-1. doi: 10.7916/D8WM1C419. Deuschl G, Bain P, Brin M; Ad hoc scientific committee. Consensusstatement of the Movement Disorder Society on Tremor. Mov Disord 1998;13:2–23. doi: 10.1002/mds.87013130310. Akakin A, Peris-Celda M, Kilic T, Seker A, Gutierrez-Martin A,Rhoton A Jr. The dentate nucleus and its projection system in the humancerebellum: the dentate nucleus microsurgical anatomical study. Neurosurgery2014;74:401–425. doi: 10.1227/NEU.000000000000029311. Krauss JK, Mohadjer M, Nobbe F, Mundinger F. The treatment ofposttraumatic tremor by stereotactic surgery. Symptomatic and functionaloutcome in a series of 35 patients. J Neurosurg 1994;80:810–819. doi: 10.3171/jns.1994.80.5.081012. Loher TJ, Krauss JK. Dystonia associated with pontomesencephaliclesions. Mov Disord 2009;24:157–167. doi: 10.1002/mds.2219613. Nieuwenhuys R, Voogd J, van Huijzen C. Funktionelle Systeme. In:Nieuwenhuys R, Voogd J, editors. Das Zentralnervensystem des Menschen,2nd ed. Berlin: Springer; 1991. p 236.Severe Bilateral Kinetic Tremor Esmaeilzadeh M, Uksul N, Krauss JKTremor and Other Hyperkinetic Movementshttp://www.tremorjournal.orgThe Center for Digital Research and ScholarshipColumbia University Libraries/Information Services3

Severe Bilateral Kinetic Tremor Due to Unilateral Midbrain Lesions

Background:&nbsp;The dentatothalamic tract connects the dentate nucleus of the cerebellum with the contralateral thalamus and plays a major role in the pathogenesis of tremor. Unilateral lesions of the dentatothalamic pathway may affect its ipsilateral predecussational or its contralateral postdecussational course, which results either in ipsilateral or in contralateral tremor.Case Report:&nbsp;Here, we present two patients with a unilateral midbrain lesion resulting in bilateral tremor. Both patients presented with severe kinetic tremor.Discussion:&nbsp;The corresponding unilateral mesencephalic lesion affected both the ipsilateral predecussational and the ipsilateral postdecussational dentatothalamic tract originating from the contralateral dentate nucleus, which is very unusual and has not been outlined clearly before.</p

Severe Bilateral Kinetic Tremor Due to Unilateral Midbrain Lesions

Abstract

Similar works

Full text

Available Versions

Columbia University Academic Commons

Directory of Open Access Journals