Painful tic convulsif caused by posterior fossa arteriovenous malformation
Niraj Kumar1, Sweety Kumari2, Saraj K Singh3, Deepak Kumar4 1 Department of Neurology, Indira Gandhi Institute of Medical Sciences, Patna, Bihar, India; Department of Neurology, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India 2 Department of Ophthalmology, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India 3 Department of Neurosurgery, All India Institute of Medical Sciences, Patna, Bihar, India 4 Department of Radiology, Indira Gandhi Institute of Medical Sciences, Patna, Bihar, India
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Correspondence Address: Dr. Niraj Kumar Department of Neurology, All India Institute of Medical Sciences, Rishikesh, Uttarakhand. India
Source of Support: None, Conflict of Interest: None
Episodes of hemifacial spasm (HFS) along with ipsilateral trigeminal neuralgia (TN) is known as painful tic convulsif (PTC). Neurovascular conflict due to vascular compression of involved cranial nerve roots is the most commonly reported pathology resulting in PTC, with ectatic course of vertebrobasilar artery being the most frequent cause. HFS and TN commonly occur in elderly population and atypical causes such as arteriovenous malformation (AVM) may likely affect young patients. To date only three cases of PTC resulting from AVM have been reported. We report a young male presenting with PTC resulting from posterior fossa AVM.
How to cite this article: Kumar N, Kumari S, Singh SK, Kumar D. Painful tic convulsif caused by posterior fossa arteriovenous malformation. Ann Mov Disord 2019;2:136-7
How to cite this URL: Kumar N, Kumari S, Singh SK, Kumar D. Painful tic convulsif caused by posterior fossa arteriovenous malformation. Ann Mov Disord [serial online] 2019 [cited 2021 Oct 18];2:136-7. Available from: https://www.aomd.in/text.asp?2019/2/3/136/272281
Acombination of ipsilateral hemifacial spasm (HFS) and trigeminal neuralgia (TN) is known as painful tic convulsif (PTC). Cushing first used the term PTC in 1920 to describe this extremely rare combination of ipsilateral HFS and TN resulting from a common pathology. Most cases are related to either an ectatic vertebrobasilar artery or posterior fossa tumor abutting the trigeminal and facial nerves., To date, only three cases of PTC resulting from arteriovenous malformation (AVM) have been reported., We report a young male presenting with PTC resulting from posterior fossa AVM.
A 20-year-old man, with noncontributory past medical and family history, presented with intermittent twitching of left half of face for the past two years and episodic painful electric sensation lasting for 3–5s in the left maxillary dermatome for the past six months. Facial twitching initially involved the left lower eyelid and gradually spread to involve left side of face and anterior neck for the past one year [Supplementary Video 1]. No history of twitching in the right half of face or any other body part was observed. Whereas the episodes of HFS occurred spontaneously, the episodes of TN were triggered on washing face with cold water or chewing. Apart from the left HFS, the detailed neurological examination failed to reveal any significant abnormality. Magnetic resonance imaging (MRI) brain revealed a posterior fossa AVM draining to vein of Galen with superior cerebellar artery, a branch of basilar, being the arterial supply. Abnormal vessels of the lesion were seen indenting the left side of the brain stem and abutting the left trigeminal and facial nerve in the left cerebellopontine cistern [Figure 1]. He refused to undergo any neurosurgical procedure for the AVM and chose botulinum toxin injection for symptomatic control. Botulinum toxin injection in a dose of 40 units at multiple sites in the left half of face improved the HFS and provided partial relief to his TN. He takes the injections every 3–4 months.
Figure 1: Magnetic resonance imaging of the brain showing abnormal dilated vascular channels of arteriovenous malformation at left cerebellopontine angle cistern and left cerebellar hemisphere indenting left vestibulocochlear and facial nerve (A, white arrow), left trigeminal nerve (B, white arrow) and indenting the brainstem and left cerebellar hemisphere (A, B and C) on axial T2-weighted images.
Neurovascular conflict due to vascular compression of involved cranial nerve roots is the most commonly reported pathology resulting in PTC, with ectatic course of vertebrobasilar artery being the most frequent cause., HFS and TN commonly occur in the elderly population as age-related brain atrophy and sinking of the brain along with elongation of brain vessels due to arteriosclerosis increase chances of approximation between cranial nerve roots and nearby vessels, thereby raising the risk of neurovascular contact., Volumetric MRI brain studies have reported lower cerebrospinal fluid volume in cases with PTC. Females usually have relatively lower cerebrospinal fluid volume and are commonly affected. Atypical causes such as AVM may likely affect young patients and males as seen in our case.
In most cases of PTC, HFS usually precedes TN as was seen in our case. Although the explanation remains uncertain, the neuroanatomical basis of this temporality has been linked to the fact that vertebrobasilar system is closer to the seventh and eighth cranial nerves as compared to the fifth cranial nerve. Therefore, the former is likely to be involved earlier. The aberrant vessels of the posterior fossa AVM in our case possibly involved the seventh cranial nerve earlier as compared to the fifth cranial nerve.
Intracranial hemorrhage may complicate approximately two-third of the posterior fossa AVM, and neurosurgical therapy including microvascular decompression, removal of AVM or its embolization is preferred.,, Percutaneous trigeminal nerve therapies including glycerol injection, thermocoagulation, or balloon compression have shown variable results for TN resulting from AVM and are not universally favored. Although botulinum toxin injection has been shown to be effective in providing symptomatic benefit in PTC, both for HFS and TN, its effect is short-lasting and needs to be repeated after every 10–12 weeks. Although reports on long-term outcome in PTC is lacking primarily due to its rarity, almost 80%–90% cure rate with a clinical stability rate of more than 80% at 10 years has been reported with microvascular decompression in cases with HFS.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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