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Abstract Details

Broken heart in Morvan’s syndrome
Autoimmune Neurology
P6 - Poster Session 6 (12:00 PM-1:00 PM)

A 50 year old lady presented with numbness in all 4 limbs along with difficulty in sleep initiation and maintenance for 3-4 months. Recently, relatives also noted intermittent abnormal behavior in patient. Patient also developed abnormal undulating movements below skin of limbs and face for one week suggestive of myokimia. She got admitted in emergency for complaints of on and off episodes of sudden onset dizziness with excessive sweating.

To present a case of Morvan’s syndrome with severe autonomic dysfunction and broken heart syndrome (Takotsubo  cardiomyopathy).

Patient also developed new form of abnormal movements in form of continuous rhythmic, tremulousness, of enough force to move small joints of limbs and even head also, suggestive of choreiform movements. Patient developed a very rapidly progressive autonomic dysfunction as fluctuations in blood pressure, cardiac rhythm abnormalities, and severe diaphoresis started developing. Electrocardiography showed ST-segment elevation in leads V1 through V3 with prolonged QT interval. Cardiac enzymes were raised, but coronary angiography was normal. Cardiac two dimensional echocardiography showed increased left ventricle diastolic diameter, apical ballooning and decreased ejection fraction, suggestive of broken heart syndrome or Takotsubo cardiomyopathy.

On evaluation, contactin associated protein-like 2 antibodies (CASPR2-Ab), a type of voltage gated potassium channel complex (Anti-VGKC) anti-bodies were detected, suggestive of Morvan’s syndrome, with normal all other relevant blood investigations. MRI brain and electro-encephalography were also normal. Patient was started on steroids, but she expired next day following cardiac arrest.

Autonomic dysfunctions are common in Morvan’s syndrome but in this case, very severe autonomic dysfuctions developed, resulting in Takotsubo cardiomyopathy leading to the death of the patient. Therefore, Morvan’s syndrome may be a cause of Takotsubo cardiomyopathy and can be considered in patients with normal coronary arteries but severe cardiac dysfunction with associated neurological symptoms.

Amit S. Singh, MD (Linux Laboratories Pvt Ltd)
Dr. Singh has nothing to disclose.
Jeenendra P. Singhvi, MD, FAAN Dr. Singhvi has nothing to disclose.
No disclosure on file
Arshdeep Singh, Jr., MBBS (House No. 869) No disclosure on file