Objective: To report a case of brainstem cavernoma associated HT and literature review.

Background: Holmes tremor is described by James Holmes in1904.Used to known as rubral,mesencephalic,thalamic tremor named according to then found anatomical correlations which characterised by irregular,unilateral,high amplitude jerks,with frequency <4.5 Hz,present at rest, exacerbated with posture, and intensified with action.¹Disruption of neuronal pathways of thalamus,midbrain,cerebellum,cerebral cortex such as dopaminergic nigrostriatal system and the cerebello-thalamo-cortical/dentate-rubro-olivary pathways are responsible.Double lesion in pathways required to manifest a Holmes Tremor,^2,3nigrostriatal pathways responsible for rest tremor,cerebello-thalamic pathways to kinetic/postural tremor.³Onset delayed for1-24 months as plasticity of brain plays a role.⁴HT is mainly due to vascular causes.cavernoma cause acute bleeds/chronic progressive neurological deficits.CT head with hyperdense lesion with calcification,MRI brain with “popcorn” lesion with hypointense rim are characteristic.^2,3.Angiographic studies can be normal due to slow flow.Unlike cavernoma of other areas of brain,Surgery is advised in patients with past bleeds targeting complete/near complete surgical resection.⁵

Method: Case report, literature review.

Results: A 43 year old woman has presented with progressive worsening of movements of left upper limb,gait imbalance, double vision for 3years.On examination she was ataxic with tendency to fall to right,gaze evoked nystagmus with dysarthria,cerebellar signs positive in upper limb.Muscle tone,deep tendon reflexes,sensory exam were normal.Left upper limb resting tremor worsen with posturing,Intension tremor had highest amplitude.CT brain shows brainstem hyperdensity and speckled calcification(Figure A).MR brain with”popcorn” appearance with rim of low signal due to hemosiderin of subclinical bleeding.(Figure B&C).Obstructive hydrocephalus was evident on scan.(Figure D).Angiography was normal.VP shunt as a temporary measure was administered.

Conclusion: Brainstem cavernoma presents acutely with bleed or with residual neurological impairment due to local effects.MRI is the investigation of choice, It may increase the risk of acute hydrocephalus by CSF drainage block which require acute surgical intervention. HT is due to disruption of control pathways in and adjacent brainstem neuronal connections.

References: 1. Bocci, T., Ardolino, G., Parenti, L., Barloscio, D., De Rosa, A., Priori, A., & Sartucci, F. (2018). Holmes’ or functional tremor? Clinical Neurophysiology Practice, 3, 104–106. 2. Raina GB, Cersosimo MG, Folgar SS, et al. Holmes tremor: Clinical description, lesion localization, and treatment in a series of 29 cases. Neurology. 2016;86(10):931–938. 3. Choi SM. Movement Disorders Following Cerebrovascular Lesions in Cerebellar Circuits. J Mov Disord. 2016;9(2):80–88. doi:10.14802/jmd.16004. 4. González Rojas N, Cesarini M, Etcheverry JL, Da Prat G, Viera Aramburu T, Gatto EM. Holmes tremor partially responsive to topiramate: a case report. Tremor Other Hyperkinet Mov. 2018; 8. 5. Li H, Ju Y, Cai Bw, Chen J, You C, Hui Xh.Experience of microsurgical treatment of brainstem cavernomas: Report of 37 cases. Neurol India 2009;57:269-73.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/holmes-tremor-ht-associated-with-brainstem-cavernous-venous-malformation-cavernoma-and-acute-hydrocephalus/