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Fantastic facial movements and where to find them 111118
1. FANTASTIC FACIAL MOVEMENTS
AND WHERE TO FIND THEM
RANDY M ROSENBERG MD FAAN FACP
ASSOCIATE PROFESSOR OF NEUROLOGY
LEWIS KATZ SCHOOL OF MEDICINE AT TEMPLE UNIVERSITY
4. TARDIVE DYSKINESIA: PHARMACOLOGIC
TREATMENT
• Clonazepam
• Ginkgo biloba
• Selective vesicular monoamine transporter 2 (VMAT2) inhibitors
• Valbenazine (Ingrezza)
• Deutetrabenazine (Austedo)
• Other therapeutic agents for which there is some anecdotal
support include vitamin E, carbidopa-levodopa),
benzodiazepines, Botox, propranolol
8. DIAGNOSTIC CRITERIA FOR TOURETTES (DSM
IV)
• Both multiple motor and one or more vocal tics have been present
at some time during the illness, although not necessarily
concurrently.
• The tics occur many times a day (usually in bouts) nearly every day
or intermittently throughout a period of more than 1 year, and
during this period there was never a tic-free period of more than 3
consecutive months
• The onset is before age 18 years.
• The disturbance is not due to the direct physiological effects of a
substance (e.g., stimulants) or a general medical condition (e.g.,
Huntington’s disease or postviral encephalitis).
10. COMORBITIES WITH TOURETTES
• The rate of co-occurrence of ADHD and TS has been reported as being
between 8 – 80%
• Suggest a shared group of genes
• Obsessive compulsive symptoms and OCD are strongly associated
with TS
• A variety of other behaviors and abnormalities have been reported to
be present in a higher than expected frequency among TS patients:
• Anxiety disorders (esp, phobias)
• Depression
• Oppositional Defiant Disorder
• Restless Leg Syndrome
• Stuttering
12. TICS AND TOURETTES: TREATMENT
PHARMACOLOGIC EXPERIMENTAL
• Deep Brain Stimulation
• Since the advent of its use in
1999, multiple targets have been
identified in DBS for TS,
including the medial thalamus,
globus pallidus internus, globus
pallidus externus, anterior limb
of the internal capsule/nucleus
accumbens, and subthalamic
nucleus
• The medial thalamus is the most
commonly reported trajectory
13. HEMIFACIAL SPASM
•Note rapid and
stereotypic
movements but not
particularly rhythmic
•Similar to synkinesia
•Don’t confuse this
with focal seizure
activity
14. HEMIFACIAL SPASM
• Segmental myoclonus of the facial nerve
• Presents in the fifth or sixth decade of life, almost always unilaterally
• The causes of hemifacial spasm
• vascular compression,
• facial nerve compression by a mass, brainstem lesions such as stroke
or
• multiple sclerosis plaques
• secondary causes such as trauma or Bell palsy
• Most instances of hemifacial spasm are probably caused by aberrant
blood vessels
• distal branches of the anterior inferior cerebellar artery or vertebral
artery compressing the facial nerve within the cerebellopontine
angle.
15. CRANIOFACIAL TREMOR
• Craniofacial tremor may occur
in association with essential
tremor, Parkinson disease,
thyroid dysfunction, or
electrolyte disturbance.
• It occurs rarely in isolation.
16. OROMANDIBULAR DYSTONIA (MEIGE
SYNDROME)
• Oromandibular dystonia
refers to dystonia affecting
the lower facial musculature,
predominantly the jaw,
pharynx, and tongue.
• When oromandibular dystonia
occurs in conjunction with
blepharospasm, the disorder
is termed Meige syndrome.
17. OROMANDIBULAR DYSTONIA (MEIGE
SYNDROME)
• Oromandibular dystonia refers to dystonia affecting the lower
facial musculature, predominantly the jaw, pharynx, and
tongue. When oromandibular dystonia occurs in conjunction
with blepharospasm, the disorder is termed Meige syndrome.
• Jaw-opening forms of oromandibular dystonia indicate primary
involvement of the digastric and lateral pterygoid.
• Jaw-closing oromandibular dystonia involves the masseter,
temporalis, and medial pterygoid.
• Jaw deviation, indicating predominant involvement of the
lateral pterygoid, is rare
20. FACIAL MYOKYMIA
• Facial myokymia appears as vermicular twitching or continuous
rippling movements under the skin, often with a wavelike spread.
• This is distinguished from other abnormal facial movements by
characteristic electromyogram discharges presenting as brief,
repetitive bursts of motor unit potentials firing at 2-60 Hz
interrupted by periods of silence of up to a few seconds.
• Facial myokymia may occur with any brainstem process.
22. SUPERIOR OBLIQUE MYOKYMIA
• Oscillation of usually one eye due to isolated firing the superior
oblique muscle
• Monocular
• Rapid
• Torsional
• Downward vertical component
• Usually no underlying pathology
• Patient can be disabled by diplopia and oscillopsia
• Treat with carbamazepine or oxcarbazepine
23. CHOVSTEK SIGN (ALONG WITH TROUSSEAU’S
SIGN)
• Clinical sign of existing nerve
hyperexcitability (tetany) seen
in hypocalcemia
• Any cause of abrupt systemic
alkalosis such as psychogenic
hyperventilation (respiratory),
or severe vomiting ( metabolic)
will lead to a fall in plasma
concentration of ionized
calcium.
• This will lead to digital and
perioral tingling, and in severe
hypocalcemia tetany and
seizures.
24. A CASE OF OCULOMASTICATORY
MYORHYTHMIA
• A 41-year-old patient developed diplopia, imbalance, and
weight loss. Examination showed pendular vergence
oscillations of the eyes and synchronous contractions of the
masticatory but not palatal muscles, i.e., oculomasticatory
myorhythmia
• There was complete supranuclear vertical and, to a lesser
extent, horizontal gaze palsy.
• The remainder of the examination was unremarkable.
• Brain MRI was normal.
25. OCULOMASTICATORY MYORHYTHMIA
Myorhythmia is defined as repetitive, rhythmic, slow (1-4 Hz) movement affecting chiefly cranial
and limb muscles. When occurring in the limbs it may be oscillatory and jerky, whereas oculo-
masticatory myorhythmia, typically associated with Whipple's disease, is a slow, repetitive, often
asymmetrical, facial and ocular movement.
26. OCULOMASTICATORY MYORHYTHMIA
• Central nervous system involvement in Whipple's disease occurs in approximately 6-43% of
patients
• Symptoms of CNS Whipple’s Disease include
• progressive dementia,
• hypothalamic dysfunction,
• myoclonus, and ocular abnormalities,
• classically a supranuclear vertical opthalmoplegia.
• OMM is usually pathognomonic of Whipple Disease.
• When present, neither jejunal biopsy nor blood or CSF PCR of Tropheryma whippelii is
necessary for the initiation of trimethoprim-sulfamethoxazole
• This patient became symptom free after 6 months of treatment.
• In addition to Whipple's disease, myorhythmia has been described in patients with
cerebrovascular disease, listeria encephalitis, anti-N-methyl-d-aspartate receptor encephalitis,
steroid-responsive encephalopathy associated with autoimmune thyroiditis, multiple sclerosis,
and other disorders.
27. FACIOBRACHIAL DYSTONIC SEIZURES IN LGI1
ENCEPHALITIS Antibodies directed to leucine-rich
glioma-inactivated 1 (LGI1) were
discovered in 2010
Mostly LGI1 resides in
hippocampus and temporal lobe
It is part of an inhibitory pathway
linking the presynaptic VGKC and
the postsynaptic a-amino-3-
hydroxy-5-methyl-4-
isoxazolepropionic acid receptor
(AMPAR)
Faciobrachial dystonic seizures have
recently been reported as
immunotherapy-responsive, brief,
frequent events that often predate
the cognitive impairment associated
with this limbic encephalitis
A 71-year-old woman presented with a 6-week history of
progressive involuntary right face and arm jerking
movements associated with prominent memory loss and
frequent falls. She subsequently developed similar
movements involving her left face and arm. Montreal
Cognitive Assessment (MoCA) score was 19/30 and physical
examination was otherwise normal. There was no alteration
of consciousness.
31. BENIGN ROLANDIC EPILEPSY
Begins between the age of 2-
10 and usually gone by
adolescence
Centrotemporal spike and
slow or sharp and slow
complexes on EEG
Editor's Notes
Seizure semiology The chapeau de gendarme or ictal pout sign is shown here during one of the patient's habitual seizures, characterized by a turned-down mouth.