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Vim thalamotomy in a patient with Holmes’ tremor and palatal tremor - Pathophysiological considerations
© Maki et al.; licensee BioMed Central. 2015
Received: 1 October 2014
Accepted: 20 February 2015
Published: 11 March 2015
We peformed a ventral intermediate nucleus (Vim) thalamotomy in a patient with Holmes’ tremor and palatal tremor. The frequencies of these movement disorders were 4 Hz and 3 Hz, respectively. Vim thalamotomy stopped the Holmes’ tremor but not the palatal tremor. Our observations suggest different mechanisms for these two involuntary movements.
A 57-arm 11 months after a pontine hemorrhage. Transoral carotid ultrasonography revealed periodic motion of her posterior pharyngeal wall with a frequency of 3 Hz. Recording of neuronal activities in the thalamus revealed a 4Hz rhythmic discharge time that was associated with her tremor in the contralateral arm. A left Vim thalamotomy was performed. The resting tremor of the upper limb stopped, but the kinetic tremor recurred 6 months after the thalamotomy. No effect was observed on her palatal tremor.
The different effects of Vim thalamotomy on the Holmes’ tremor and palatal tremor suggest different oscillation sources for these two involuntary movements.
Holmes’ tremor is defined by the Ad Hoc Scientific Committee of the Movement Disorder Society as a general term for symptomatic tremors, including midbrain tremors, rubral tremors, thalamic tremors, and tremors in Benedict’s syndrome . Characteristics of Holmes’ tremors include that they <1 > have a delayed onset from 4 weeks to 2 years after a stroke; <2 > are low-frequency (≤4.5 Hz) tremors primarily of proximal muscles; and <3 > are rest, action, and postural tremors that increase with movement. Reported foci include the area superolateral to the red nuclei, rubrothalamic tract, central tegmental tract, superior cerebellar peduncle, and the substantia nigra . Drug therapy including L-dopa, anticholinergic agents, and zonisamide is effective in some cases . This suggests dysfunction of the nigrostriatal tract , which is thought to be involved in the onset of rest tremors.
Moreover, in patients with midbrain tremors, 2β-Carbomethoxy-3β-(4-(123) I-iodophenyl) tropane ((123) I-beta-CIT) SPECT shows decreased uptake in the putamen and caudate nucleus on the side of the lesion, thus suggesting a relationship between these sites and symptoms . These same foci are also involved in palatal tremor, but the pathogenesis varies among patients. Many cases are resistant to oral drug therapy, and the pathophysiology is unclear. We recently performed a ventral intermediate nucleus (Vim) thalamotomy in a patient with Holmes’ tremor and palatal tremor, and neuronal activity in the thalamus was observed. We now discuss the pathophysiology in this case based on characteristic findings.
A 57-year-old right-handed woman with hypertension suddenly developed right hemiparesis and ataxia in 2010. Eleven months after the left pontine tegmentum hemorrhage, she developed a resting tremor in her right upper extremity. Trihexyphenidyl HCl, levodopa/carbidopa hydrate, and arotinolol were prescribed for outpatient use but were not clearly effective. Thus, 2 years after the cerebral hemorrhage, she was admitted to the hospital for surgical treatment. On admission, no abnormal findings were seen with a general physical examination. The patient was lucid but had ataxic dysarthria and palatal tremor. She also had mild right hemiplegia and a cerebellar ataxic gait. A coarse tremor was present, primarily of the proximal right upper extremity at rest and with posture that increased with movement. The deep tendon reflexes were normal, but superficial sensation in the right upper extremity was decreased.
The resting tremor stopped during the month after surgery, almost no action tremor was observed, and the patient was able to practice handwriting. Subsequently, the action tremor again gradually appeared. On evaluation after 6 months, no resting tremor was seen, but about a 2.5Hz (slower than before surgery) coarse tremor with posture and movement was present. The palatal tremor remained unchanged compared to before surgery. The CRST score increased from 20 to 42, and subtotal scores of Part A, B and C were 7, 15, and 20, respectively.
Damage to the cerebellothalamic tract is thought to be involved in Holmes’ action tremors .Our patient also had disruption of afferent pathways (deafferentation) from the cerebellar dentate nucleus to the thalamus due to a brainstem lesion, which presumably led to thalamic functional changes. In other words, this suggests that the approximately 4-Hz rhythmic discharges observed in the thalamus during surgery that corresponded to the tremor rhythm contributed to the development of the tremor.
Meanwhile, palatal tremor is due to disruption of pathways from the dentate nucleus to the inferior olivary nucleus via the central tegmental tract, and is caused by intrinsic spontaneous firing of cells in the inferior olivary nucleus . Inhibitory fibers from the contralateral cerebellum are usually distributed to gap junctions between inferior olivary nucleus cells, and this inhibits electrotonic coupling between cells . When lesions occur as in our patient, the cerebellar inhibitory tracts are disrupted, inhibition of electrotonic coupling is lost, and synchronous rhythmic discharges occur [8,9]. A similar oscillatory mechanism, as in the inferior olivary nucleus, is also thought to exist in the thalamus .
Surgical treatment for Holmes’ tremors includes thalamotomy targeting the thalamic Vim [11,12], DBS [13-16], and a combination of subthalamic nucleus DBS . However, these treatments are often only partially effective, including cases with improvement in only distal and action tremors, and other cases with a short duration of effectiveness. For proximal tremors in which control with Vim-DBS has been difficult, coagulation of the internal segment of the globus pallidus is reported to be effective [18,19]. Globus pallidus output may also be involved in Holmes’ tremors, and since 2000, the effectiveness of zona incerta DBS has been gaining attention .Resolution of palatal tremor, however, has not been described in these reports [12,18].
We performed a Vim thalamotomy in a patient with Holmes’ tremor and palatal tremor after a brainstem hemorrhage. Using semi-microelectrode recordings, rhythmic discharges corresponding to the tremor rhythm were observed in a wide area around the Vim nucleus of the thalamus, suggesting involvement of this site in the upper extremity tremors. The tremors temporarily resolved after coagulation of these thalamic neurons, but they recurred in a few months, and thus, the improvement was limited. These types of functional changes in thalamic neurons over a wide area, cerebellar ataxia, and the existence of multiple independent sites of origin can make treatment difficult.
Written informed consent was obtained from the patient for the publication of this case report and accompanying images.
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