Case Report: Brugada Syndrome - A Cardiac Channelopathy.
Poster used for presentation in CMC MAC 2021.
OBJECTIVE: To discuss an interesting case of Brugada syndrome presenting as seizures.
BACKGROUND: A 25-year-old well-informed male presented to us with complaints of seizure on day 3 of an acute febrile illness. He was conscious, oriented, GCS15/15 and system examinations were unremarkable. He had a similar history of seizure during fever 1 year back and was started on anti-epileptics since then and was treated with empirical antibiotics and CSF analysis, MRI brain with seizure protocol and EEG were completely normal during that episode. As described by patient, both episodes were very similar and was like darkening of visual field followed by LOC and bystanders witnessed few jerks involving both sides of body followed by regaining consciousness. This raised suspicion for syncope and ECG revealed RBBB-rSR’ pattern and saddleback STE in V1-V3(type2-brugada pattern-not diagnostic on its own). But on probing, patient revealed SCD in his father at age 42.
RESULTS: Echo revealed structurally normal heart. Expert opinion sought and flecainide challenge test revealed the classical type1 brugada pattern (diagnostic) with coved STE and T inversion in V1-V3 clinching the diagnosis of BRUGADA SYNDROME. Genetic testing for channelopathy was unremarkable. Type 1 Brugada pattern (on provocative testing) along with syncopal event and family history strongly warranted AICD insertion and patient opted for subcutaneous ICD. 6 months later, ICD interrogation revealed occurrence of 1 episode of NSVT, which fell below the ICD intervention threshold.
CONCLUSION: Brugada syndrome is a rare cardiac channelopathy with high risk of SCD in the absence of intervention. Events during fever and family history are very classical. It has male preponderance and more seen in Southeast Asia. All cases of suspected syncopal attacks warrant a thorough search for ECG markers of SCD.
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Brugada Syndrome - A Cardiac Channelopathy: Case Report
1. Dr. Manievelraaman Kannan, Junior Resident, Institute of Internal Medicine,
Madras Medical College & Rajiv Gandhi Government General Hospital, Chennai – 03.
CLINICAL PRESENTATION
WORKUP
CONCLUSION
DIAGNOSIS & MANAGEMENT
REFERENCES
Figure 1. Baseline ECG showing Type 2
Brugada pattern
Figure 2. Type 1 Brugada pattern
INTRODUCTION
CONTACT
BRUGADA SYNDROME
It’s just ‘NOT SEIZURES’ – It’s Brugada Syndrome (BrS)
Email: drmvraaman1994@outlook.com
Phone: 9789999778
Cardiac Electrophysiology consult was obtained and as suggested, patient underwent Flecainide challenge test.
Flecainide challenge revealed classical Type 1 Brugada pattern (coved ST segment elevation and T inversion in V1-V2)
in ECG, which by itself is very significant.
Genetic testing for cardiac channelopathy was unremarkable
Type 1 Brugada pattern (on provocative testing) along with
history of syncopal events and family history of sudden
cardiac death strongly suggested the diagnosis of Brugada
syndrome and it also warranted the insertion of ICD.
Patient advised to avoid all medications, including OTC
medications. Patient counselled about the therapy and the
need for therapy. Patient opted for subcutaneous AICD
insertion and the procedure was uneventful.
Reviewing 6 months after AICD placement, during ICD
interrogation, it was found that he had one episode of NSVT
which converted to normal sinus rhythm spontaneously.
CBC, RFT, LFT, Electrolytes – Normal
Fever profile: (MP/MF, Dengue, Widal, MSAT, Scrub
IgM- negative
ECG – rSR’ pattern in V1 with saddleback STE in V1-V2
(Correct lead placements confirmed)
Echo: Normal
Investigations during last episode:
CT brain – normal
CSF analysis – normal
MRI brain with seizure protocol – normal
EEG – normal
In this case, ECG changes, events during fever episodes, and
family history are very classical of Brugada syndrome.
Incorrect lead placements and chest wall deformities should
be ruled out as it may sometimes mimic Brugada pattern in
ECG.
All cases of suspected seizure / syncopal attacks warrant a
thorough search for ECG markers of SCD, of which BrS is one
rare differential diagnosis.
A 25-year-old male presented to us with complaints
of seizure on day 3 of an acute febrile illness.
No complaints of headache, visual disturbances,
vomiting and neck stiffness.
He had a similar episode of seizure during fever
one year back and was treated with empirical
antibiotics and started on anti-epileptics since then.
CSF analysis, MRI brain with seizure protocol and
EEG were completely normal during that episode.
He has no other co-morbidities.
On examination, he was conscious, oriented,
afebrile, GCS 15/15, hemodynamically stable and
was having no signs of meningeal irritation. System
examinations (CVS, RS, Abdomen, CNS) were
unremarkable.
As described by the patient, both episodes were
very similar and was like darkening of visual field
followed by LOC and bystanders witnessed few
jerks involving both sides of body followed by
regaining of consciousness spontaneously. This
description raised the suspicion for syncope.
ECG revealed normal sinus rhythm, rSR’ pattern in V1
and saddleback ST segment elevation in V1-V2. This can
be read as Type 2 Brugada pattern of ECG, which by
itself has no much significance. But, on probing, patient
revealed sudden cardiac death (SCD) in his father
at age 42. Hence, patient is evaluated further.
1. Epilepsy and brugada syndrome: Association or uncommon presentation?
https://www.heartviews.org/text.asp?2020/21/2/114/288327
2. Electrical Storm in the Brain and in the Heart: Epilepsy and Brugada Syndrome
https://doi.org/10.1016/j.mayocp.2013.06.019
3. A Fatal Case of Mistaken Identity: Brugada Syndrome Masquerading as Seizure
Disorder (P2.264) Claribel Wee, Julius Latorre Neurology Apr 2018, 90 (15
Supplement) P2.264
Subcutaneous ICD device
Brugada Syndrome is a rare cardiac
channelopathy known to cause
malignant ventricular arrhythmias
and sudden cardiac death —
especially during sleep in young
otherwise healthy males, in South
East Asian region, where it’s known
by various names.
However, in many cases, such
catastrophe can be alleviated with
appropriate interventions.
The importance lies in making the
prompt diagnosis, which in some
cases can be delayed or confounded,
like we experienced in this case.
With this background, here’s a case
report of Brugada syndrome
masquerading as seizure disorder.
Type 1 Brugada Pattern
(in baseline ECG or on provocative testing)
PLUS
EVENTS (OR) FAMILY HISTORY
Polymorphic VT/VF
Unexplained syncope
Nocturnal agonal
respiration
SCD (<45 year old)
Relative with Type 1
Brugada pattern ECG
Normal EEG & CT brain report
Figure 3. Subcutaneous ICD device