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Myoclonic triangle

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Labeled diagram showing some of the cerebellar neural tracts. Only the nucleus ruber and the dentate nucleus are shown in this diagram; the olivary nucleus is positioned on the lateral aspect of the brainstem.
Palatal myoclonus

The myoclonic triangle (also known by its eponym Triangle of Guillain-Mollaret or dentato-rubro-olivary pathway) is an important feedback circuit of the brainstem and deep cerebellar nuclei which is responsible for modulating spinal cord motor activity.

The circuit is thus composed:

  1. Fibers of the rubro-olivary tract project from the parvocellular red nucleus via the central tegmental tract to the ipsilateral inferior olivary nucleus.
  2. The inferior olivary nucleus sends its afferents via climbing fibers in the inferior cerebellar peduncle to Purkinje cells of the contralateral cerebellar cortex.
  3. The Purkinje cells send their afferents to the ipsilateral dentate nucleus.
  4. Dentatorubral tract fibers: the dentate nucleus afferents travel via the superior cerebellar peduncle to the contralateral red nucleus, thus completing the circuit.

Of note, this circuit contains a double decussation, implying that a lesion in this tract will cause ipsilateral symptoms.

The descending rubrospinal tract and reticulospinal tract originate in the red nucleus and reticular formation (which is closely associated with the central tegmental tract) respectively, thereby providing the mechanism by which this circuit exerts its effects on spinal cord motor activity.

Pathologies

Hypertrophic olivary degeneration

HOD is caused by lesions in the dentatorubral or central tegmental tracts. Lesions of the superior cerebellar peduncle can also result in contralateral HOD, whereas primary lesions of the central tegmental tract cause ipsilateral HOD. Lesions involving this circuit may produce palatal myoclonus, one of the few involuntary movements that do not disappear during sleep. Palatal myoclonus may be seen as a component of the lateral medullary syndrome (a.k.a. Wallenberg Syndrome), if the infarction extends to involve the central tegmental tract.

Holmes tremor

Descriptions of Holmes tremor associated with HOD are scarce. It is most likely that disruption of the disynaptic dentate-rubro-olivary tract degeneration is associated with tremor and disruption of the monosynaptic dentate-olivary tract is associated with HOD. The convergence of both components makes the combination of Holmes tremor and HOD after upper brainstem damage plausible and even likely.

References

  1. Murdoch, Sheena; Shah, Pushkar; Jampana, Ravi (June 2016). "The Guillain-Mollaret triangle in action". Practical Neurology. 16 (3): 243–246. doi:10.1136/practneurol-2015-001142. ISSN 1474-7766. PMID 26740379. S2CID 207025040.
  2. ^ Lavezzi, Anna Maria; Corna, Melissa; Matturri, Luigi; Santoro, Franco (2009-07-01). "Neuropathology of the Guillain-Mollaret Triangle (Dentato-Rubro-Olivary Network) in Sudden Unexplained Perinatal Death and SIDS". The Open Neurology Journal. 3 (1): 48–53. doi:10.2174/1874205X00903010048. ISSN 1874-205X. PMC 2708385. PMID 19597559.
  3. Cosentino, Carlos; Velez, Miriam; Nuñez, Yesenia; Palomino, Henry; Quispe, Darko; Flores, Martha; Torres, Luis (2016-07-15). "Bilateral Hypertrophic Olivary Degeneration and Holmes Tremor without Palatal Tremor: An Unusual Association". Tremor and Other Hyperkinetic Movements. 6: 400. doi:10.7916/D87944SS. ISSN 2160-8288. PMC 4954943. PMID 27536461.
  4. Khoyratty, Fadil; Wilson, Thomas (2013). "The Dentato-Rubro-Olivary Tract: Clinical Dimension of This Anatomical Pathway". Case Reports in Otolaryngology. 2013: 934386. doi:10.1155/2013/934386. ISSN 2090-6765. PMC 3639700. PMID 23662232.
  5. Raina, Gabriela B.; Cersosimo, Maria G.; Folgar, Silvia S.; Giugni, Juan C.; Calandra, Cristian; Paviolo, Juan P.; Tkachuk, Veronica A.; Zuñiga Ramirez, Carlos; Tschopp, Andrea L. (2016-03-08). "Holmes tremor". Neurology. 86 (10): 931–938. doi:10.1212/WNL.0000000000002440. ISSN 0028-3878. PMC 4782118. PMID 26865524.
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