Vim thalamotomy in a patient with Holmes’ tremor and palatal tremor - Pathophysiological considerations

Hypertrophic olivary degeneration (HOD) is a rare type of neuronal degeneration involving the dento-rubro-olivary pathway and presents clinically as palatal tremor. We present a 48 year old male patient who developed Holmes' tremor and bilateral HOD five months after brainstem hemorrhage. The severe rest tremor was refractory to pharmacotherapy and botulinum toxin injections, but was markedly reduced after thalamotomy. Magnetic resonance imaging permitted visualization of HOD, which appeared as a characteristic high signal intensity in the inferior olivary nuclei on T2- and proton-density-weighted images. Enlargement of the inferior olivary nuclei was also noted. Palatal tremor was absent in that moment and appears about two months later. The delayed-onset between insult and tremor following structural lesions of the brain suggest that compensatory or secondary changes in nervous system function must contribute to tremor genesis. The literature and imaging findings of this uncommon condition are reviewed.

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Multicenter Prospective Analysis of Hypertrophic Olivary Degeneration Following Infratentorial Stroke (HOD-IS): Evaluation of Disease Epidemiology, Clinical Presentation, and MR-Imaging Aspects

Frontiers in Neurology ◽

10.3389/fneur.2021.675123 ◽

2021 ◽

Vol 12 ◽

Author(s):

Martin A. Schaller-Paule ◽

Eike Steidl ◽

Manoj Shrestha ◽

Ralf Deichmann ◽

Helmuth Steinmetz ◽

...

Keyword(s):

Mr Imaging ◽

Clinical Symptoms ◽

Inferior Olivary Nucleus ◽

Index Event ◽

Hypertrophic Olivary Degeneration ◽

Palatal Tremor ◽

Prophylactic Measures ◽

Holmes Tremor ◽

Dti Tractography ◽

Tractography Data

Introduction: Ischemic and hemorrhagic strokes in the brainstem and cerebellum with injury to the functional loop of the Guillain-Mollaret triangle (GMT) can trigger a series of events that result in secondary trans-synaptic neurodegeneration of the inferior olivary nucleus. In an unknown percentage of patients, this leads to a condition called hypertrophic olivary degeneration (HOD). Characteristic clinical symptoms of HOD progress slowly over months and consist of a rhythmic palatal tremor, vertical pendular nystagmus, and Holmes tremor of the upper limbs. Diffusion Tensor Imaging (DTI) with tractography is a promising method to identify functional pathway lesions along the cerebello-thalamo-cortical connectivity and to generate a deeper understanding of the HOD pathophysiology. The incidence of HOD development following stroke and the timeline of clinical symptoms have not yet been determined in prospective studies—a prerequisite for the surveillance of patients at risk.Methods and Analysis: Patients with ischemic and hemorrhagic strokes in the brainstem and cerebellum with a topo-anatomical relation to the GMT are recruited within certified stroke units of the Interdisciplinary Neurovascular Network of the Rhine-Main. Matching lesions are identified using a predefined MRI template. Eligible patients are prospectively followed up and present at 4 and 8 months after the index event. During study visits, a clinical neurological examination and brain MRI, including high-resolution T2-, proton-density-weighted imaging, and DTI tractography, are performed. Fiberoptic endoscopic evaluation of swallowing is optional if palatal tremor is encountered.Study Outcomes: The primary endpoint of this prospective clinical multicenter study is to determine the frequency of radiological HOD development in patients with a posterior fossa stroke affecting the GMT at 8 months after the index event. Secondary endpoints are identification of (1) the timeline and relevance of clinical symptoms, (2) lesion localizations more prone to HOD occurrence, and (3) the best MR-imaging regimen for HOD identification. Additionally, (4) DTI tractography data are used to analyze individual pathway lesions. The aim is to contribute to the epidemiological and pathophysiological understanding of HOD and hereby facilitate future research on therapeutic and prophylactic measures.Clinical Trial Registration: HOD-IS is a registered trial at https://www.drks.de/drks_web/navigate.do?navigationId=trial.HTML&TRIAL_ID=DRKS00020549.

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Successful Therapy of Holmes Tremor with a Silicone Hand-Wrist Orthesis and Botulinum Toxin A

Neuropediatrics ◽

10.1055/s-0036-1583680 ◽

2016 ◽

Vol 47 (S 01) ◽

Author(s):

Th. Becher ◽

A. Hägele ◽

S. Weise

Keyword(s):

Botulinum Toxin ◽

Botulinum Toxin A ◽

Successful Therapy ◽

Toxin A ◽

Holmes Tremor

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Holmes tremor: a delayed complication after resection of brainstem cavernomas

Journal of Neurosurgery ◽

10.3171/2020.7.jns201352 ◽

2020 ◽

pp. 1-11

Author(s):

Marco Cenzato ◽

Davide Colistra ◽

Giorgia Iacopino ◽

Christian Raftopoulos ◽

Ulrich Sure ◽

...

Keyword(s):

Statistical Analysis ◽

Neurophysiological Monitoring ◽

Strict Adherence ◽

Surgical Access ◽

Holmes Tremor ◽

Neurophysiological Studies ◽

Metropolitan Hospital ◽

Delayed Complication ◽

Brainstem Cavernoma ◽

Predicting Factor

OBJECTIVEIn this paper, the authors aimed to illustrate how Holmes tremor (HT) can occur as a delayed complication after brainstem cavernoma resection despite strict adherence to the safe entry zones (SEZs).METHODSAfter operating on 2 patients with brainstem cavernoma at the Great Metropolitan Hospital Niguarda in Milan and noticing a similar pathological pattern postoperatively, the authors asked 10 different neurosurgery centers around the world to identify similar cases, and a total of 20 were gathered from among 1274 cases of brainstem cavernomas. They evaluated the tremor, cavernoma location, surgical approach, and SEZ for every case. For the 2 cases at their center, they also performed electromyographic and accelerometric recordings of the tremor and evaluated the postoperative tractographic representation of the neuronal pathways involved in the tremorigenesis. After gathering data on all 1274 brainstem cavernomas, they performed a statistical analysis to determine if the location of the cavernoma is a potential predicting factor for the onset of HT.RESULTSFrom the analysis of all 20 cases with HT, it emerged that this highly debilitating tremor can occur as a delayed complication in patients whose postoperative clinical course has been excellent and in whom surgical access has strictly adhered to the SEZs. Three of the patients were subsequently effectively treated with deep brain stimulation (DBS), which resulted in complete or almost complete tremor regression. From the statistical analysis of all 1274 brainstem cavernomas, it was determined that a cavernoma location in the midbrain was significantly associated with the onset of HT (p < 0.0005).CONCLUSIONSDespite strict adherence to SEZs, the use of intraoperative neurophysiological monitoring, and the immediate success of a resective surgery, HT, a severe neurological disorder, can occur as a delayed complication after resection of brainstem cavernomas. A cavernoma location in the midbrain is a significant predictive factor for the onset of HT. Further anatomical and neurophysiological studies will be necessary to find clues to prevent this complication.

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Psychogenic palatal tremor may be underrecognized: Reappraisal of a large series of cases

Movement Disorders ◽

10.1002/mds.24948 ◽

2012 ◽

Vol 27 (9) ◽

pp. 1164-1168 ◽

Cited By ~ 31

Author(s):

Maria Stamelou ◽

Tabish A. Saifee ◽

Mark J. Edwards ◽

Kailash P. Bhatia

Keyword(s):

Large Series ◽

Palatal Tremor

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Function of the cerebellum in parkinsonian rest tremor and Holmes' tremor

Annals of Neurology ◽

10.1002/1531-8249(199907)46:1<126::aid-ana20>3.0.co;2-3 ◽

1999 ◽

Vol 46 (1) ◽

pp. 126-128 ◽

Cited By ~ 55

Author(s):

G. Deuschl ◽

H. Wilms ◽

P. Krack ◽

M. W�rker ◽

W.-D. Heiss

Keyword(s):

Rest Tremor ◽

Holmes Tremor

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Possible necessity for deep brain stimulation of both the ventralis intermedius and subthalamic nuclei to resolve Holmes tremor

Journal of Neurosurgery ◽

10.3171/jns.2003.99.3.0566 ◽

2003 ◽

Vol 99 (3) ◽

pp. 566-571 ◽

Cited By ~ 49

Author(s):

Pantaleo Romanelli ◽

Helen Bronté-Stewart ◽

Tracy Courtney ◽

Gary Heit

Keyword(s):

Deep Brain Stimulation ◽

Brain Stimulation ◽

Clinical Observation ◽

Intention Tremor ◽

Content Type ◽

Resting Tremor ◽

Holmes Tremor ◽

Tremor Analysis ◽

Deep Brain ◽

Stimulation Of

✓ Holmes tremor is characterized by resting, postural, and intention tremor. Deep brain stimulation (DBS) of both the nucleus ventralis intermedius (Vim) and the subthalamic nucleus (STN) may be required to control these three tremor components. A 79-year-old man presented with a long-standing combination of resting, postural, and intention tremor, which was associated with severe disability and was resistant to medical treatment. Neuroimaging studies failed to reveal areas of discrete brain damage. A DBS device was placed in the Vim and produced an improvement in both the intention and postural tremor, but there was residual resting tremor, as demonstrated by clinical observation and quantitative tremor analysis. Placement of an additional DBS device in the STN resolved the resting tremor. Stimulation of the Vim or STN alone failed to produce global resolution of mixed tremor, whereas combined Vim—STN stimulation produced global relief without creating noticeable side effects. Combined Vim—STN stimulation can thus be a safe and effective treatment for Holmes tremor.

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Palatal tremor and orofacial stereotypies within the locked-in syndrome

Movement Disorders ◽

10.1002/mds.21327 ◽

2007 ◽

Vol 22 (4) ◽

pp. 593-595 ◽

Cited By ~ 3

Author(s):

Philip Duffey ◽

Neil Jenkins ◽

David King

Keyword(s):

Palatal Tremor

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