Treatment-responsive Holmes tremor in a child with low-pressure hydrocephalus: video case report and systematic review of the literature

Stephano J. Chang MD, PhD1, Ruth Mitchell BA, BSc, BMBS2, Juliette Hukin MBBS3, and Ash Singhal MD, MSc2
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  • 1 Division of Neurosurgery, Department of Surgery, University of British Columbia;
  • | 2 Faculty of Medicine and the Division of Neurosurgery, University of British Columbia and British Columbia Children’s Hospital; and
  • | 3 Divisions of Neurology and Hematology/Oncology/Bone Marrow Transplant, Department of Pediatrics, University of British Columbia and British Columbia Children’s Hospital, Vancouver, British Columbia, Canada
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OBJECTIVE

Holmes tremor (HT) is a rare and debilitating movement disorder comprising both rest and action tremor, and it is known for its resistance to treatment. Its most common causes include ischemic or hemorrhagic insults and trauma. Mechanistically, the combined rest and action tremor is thought to require a double lesion of both the dopaminergic nigrostriatal system and the dentatorubrothalamic pathways, often near the midbrain where both pathways converge. The aim of this study was to characterize HT as a presenting sign in cases of hydrocephalus and to discuss potential pathomechanisms, clinical presentations, and treatment options.

METHODS

MEDLINE and Web of Science were searched for cases of HT with hydrocephalus from database inception to August 2021, and these were compiled along with the authors’ own unique case of treatment-responsive HT in a child with low-pressure obstructive hydrocephalus secondary to a tectal tumor. Patient characteristics, presenting signs/symptoms, potential precipitating factors, interventions, and patient outcomes were recorded.

RESULTS

Nine patients were identified including the authors’ video case report. All patients had a triventriculomegaly pattern with at least a component of obstructive hydrocephalus, and 4 patients were identified as having low-pressure hydrocephalus. Parinaud’s syndrome and bradykinesia were the most commonly associated signs. Levodopa and CSF diversion were the most commonly used and effective treatments for HT in this population. This review was not registered and did not receive any funding.

CONCLUSIONS

HT is a poorly understood and probably underrecognized presentation of hydrocephalus that is difficult to treat, limiting the strength of the evidence in this review. Treatment options include CSF diversion, antiparkinsonian agents, antiepileptic agents, deep brain stimulation, and MR-guided focused ultrasound, and aim toward the nigrostriatal and dentatorubrothalamic pathways hypothesized to be involved in its pathophysiology.

ABBREVIATIONS

DBS = deep brain stimulation; DRTT = dentatorubrothalamic tract; ETV = endoscopic third ventriculostomy; HT = Holmes tremor; ICP = intracranial pressure; NST = nigrostriatal tract; STN = subthalamic nucleus; Vim = ventral intermediate nucleus; VPS = ventriculoperitoneal shunt.

Diagram from Behbahani et al. (pp 488–496).

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