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LETTER TO EDITOR
Year : 2021  |  Volume : 69  |  Issue : 2  |  Page : 515--516

Paroxysmal Dysarthria Ataxia–Tremor–Blepharospasm Syndrome in Bickerstaff Brainstem Encephalitis: A Variant of Paroxysmal Dysarthria Ataxia Syndrome

Enrico Ferrante1, Michele Trimboli2,  
1 Department of Neurology, AOR San Carlo, Potenza; Department of Neurology, Alto Vicentino Hospital - AULSS 7 Pedemontana, Santorso, Italy
2 Department of Neurology, AOR San Carlo, Potenza; Department of Neuroscience, S. Giovanni di Dio Hospital, Crotone, Italy

Correspondence Address:
Enrico Ferrante
Department of Neurology, Alto Vicentino Hospital - AULSS 7 Pedemontana, Santorso (VI)
Italy




How to cite this article:
Ferrante E, Trimboli M. Paroxysmal Dysarthria Ataxia–Tremor–Blepharospasm Syndrome in Bickerstaff Brainstem Encephalitis: A Variant of Paroxysmal Dysarthria Ataxia Syndrome.Neurol India 2021;69:515-516


How to cite this URL:
Ferrante E, Trimboli M. Paroxysmal Dysarthria Ataxia–Tremor–Blepharospasm Syndrome in Bickerstaff Brainstem Encephalitis: A Variant of Paroxysmal Dysarthria Ataxia Syndrome. Neurol India [serial online] 2021 [cited 2021 Jun 24 ];69:515-516
Available from: https://www.neurologyindia.com/text.asp?2021/69/2/515/314518


Full Text



Sir,

We read with interest the article by Maramattom.[1]

This article reported a case of Bickerstaff encephalitis presenting paroxysmal episodes lasting for <1 min and characterized by side to side head tremor, head tilt to the left, dysarthria, along with blepharospasm. MRI showed a symmetric midbrain lesion. Paroxysmal Dysarthria Ataxia–Tremor–Blepharospasm Syndrome was diagnosed and classified as variant of Paroxysmal Dysarthria Ataxia (PDA) syndrome.[2] This new syndrome, as described cases of PDA, well responded to carbamazepine. Furthermore, the author rightly stated PDA syndrome may remit spontaneously in MS.

We noted that an ictal video-EEG to exclude the possible epileptic nature of the paroxysmal attacks was not performed in this patient.[3] Moreover, a brain MRI follow-up to monitor any causative lesion changes after symptoms resolution have not been done.

We have recently described a similar case as variant of PDA Syndrome.[4]

A 67-year-old man developed paroxysmal ataxia, dysarthria (distorted voice that sounded like a 45-rpm record played at 33 rpm), diplopia, and hemifacial spasms episodes lasting for 15–40 s and occurring ~40 times/day. Ictal video-EEGs did not show paroxysmal discharges. Brain MRI revealed a right-midbrain lesion by Neuro-Behçet's disease. PADDHS attacks lasted for 5 months and then disappeared, and brain MRI returned to normal.

In our opinion, since the number and intensity of episodes described by Maramattom reduced by >90% after 1-week treatment with an antiepileptic drug (Carbamazepine 600 mg a day), a video-EEG, to exclude the possible epileptic nature of the paroxysmal attacks, should have been performed.

In conclusion, our case showed that spontaneous resolution of PAD attacks are also described in Neuro-Behçet's disease; furthermore, it demonstrates that a temporal relationship between symptoms resolution and brain MRI causative lesion disappearance should be sought.

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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1Maramattom BV. Paroxysmal dysarthria ataxia-tremor-blepharospasm syndrome in bickerstaff brainstem encephalitis: A variant of paroxysmal dysarthria ataxia syndrome. Neurol India 2019;67:1388-9.
2Gorard DA, Gibberd FB. Paroxysmal dysarthria and ataxia: Associated MRI abnormality. J Neurol Neurosurg Psychiatry 1989;52:1444-5.
3Lüders H, Vaca GF, Akamatsu N, Amina S, Arzimanoglou A, Baumgartner C, et al. Classification of paroxysmal events and the four-dimensional epilepsy classification system. Epileptic Disord 2019;21:1-29.
4Ferrante E, Marazzi MR, Trimboli M, Dalla Costa D, Erminio C, Nobili L. Brainstem lesion causing paroxysmal ataxia, dysarthria, diplopia and hemifacial spasm (PADDHS). Epileptic Disord 2019;21:389-90.