Guillain-Mollaret triangle

Anatomy of the triangle of Guillain

and Mollaret
• Red nucleus (RN)
• Contralateral dentate nucleus (DN)
of cerebellum
• ION ipsilateral to RN

* Tract connections:

•Central tegmental: connects RN to ipsilateral ION

•Superior cerebellar peduncle (dentatorubral
tract): connects DN to contralateral RN
 Physiopathology
•1st: Brain stem insults that may lead to
dentatorubral-olivary pathway interruption as:
hemorrhage, ischemic infarction, demyelination
or tumors.
•2nd: Olivary de-afferentation.
•3rd: Enlargement rather than atrophy of the
affected structure (unique finding in this form of
transneuronal degeneration).
 Clinical Issues
•Most common signs and symptoms are:

–Symptomatic palatal tremor/myoclonus: Rhythmic

involuntary movement of soft palate, uvula, pharynx, and
larynx.
–Severe myoclonus of cervical muscles and diaphragm
–Dentatorubral tremor (Holmes' tremor): postural and
kinetic tremor of an upper extremity.
–Symptoms of cerebellar or brain stem dysfunction
• Damage to GMT areas or the connections
between them can cause oculopalatal tremor.
The cerebellum is predominantly excitatory, but
the dentate nucleus within it has the primary
function of inhibiting the inferior olivary nucleus
via GABAergic projections. If the triangle is
interrupted by pathology (vascular insult,
demyelination or tumour), the inferior olivary
nucleus is no longer inhibited and it
hypertrophies. The nucleus contains many gap
junctions that rhythmically discharge at 0.5– 3.0
Hz, causing oculopalatal tremor at that frequency
• Olivary nucleus hypertrophy can occur
without the patient developing an ocular or
palatal tremor.
 OCULOPALATAL TREMOR
• The ocular nystagmus can be vertical,
horizontal or torsional. It is described as
‘pendular’, meaning the eye movements are
smooth with equal speed in both directions,
unlike jerk nystagmus where the deviation
phase is slow and the corrective phase is fast.
The frequency of the ocular tremor is 1–3 Hz
and is in tandem with the palatal tremor.
• The palatal tremor is rhythmic and patients
may complain of hearing a clicking sound. It
does not usually cause difficulty with speech
or swallowing. Unlike other movement
disorders, palatal tremor continues during
sleep, probably because it is brainstem-
mediated, rather than from pathology in the
basal ganglia.