A RETROSPECTIVE STUDY ON NEUROPSYCHOLOGICAL AND AUTONOMIC OBSERVATIONS OF CASPR2-RELATED MORVAN SYNDROME.

Rashmi  Singh; Nikhil  Kumar

doi:10.51168/sjhrafrica.v5i3.1006

Authors

Rashmi Singh PG, Department of General Medicine, I.G.I.M.S., Patna, Bihar, India.
Nikhil Kumar Junior Resident, Department of Neuro Medicine, I.G.I.M.S., Patna, Bihar, India.

DOI:

https://doi.org/10.51168/sjhrafrica.v5i3.1006

Keywords:

Morvan Syndrome, Autoimmune encephalitis, Autonomic dysfunction, CASPR2

Abstract

Introduction:

The defining feature of Morvan syndrome is cerebral, autonomic, and peripheral hyperexcitability; this is caused by the antibody against contactin-associated protein 2 (CASPR2). The study aimed to examine the cognitive, autonomic, electrophysiologic, polysomnographic, and clinical spectrum of Morvan syndrome patients associated with CASPR2.

Methods:

A retrospective study was conducted. Samples of serum and CSF positive for CASPR2 antibodies for three years were evaluated. Those with Morvan syndrome, identified by clinical and electrophysiologic basis, were among them.

Results:

Among the patients with Morvan syndrome, 28 (M: F = 10:4) had an onset of 37.1 ± 17.5 years. Clinical characteristics included spastic speech (4), dysphagia (4), behavioral abnormalities (4), seizures (2), cold intolerance (2), muscular twitching (24), sleeplessness (24), pain (22), paresthesias (18), hyperhidrosis (14), hypersalivation (12), double incontinence (6). Myokymia (24), hyperactive tendon reflexes (20), and tremor (12) were found during the neurologic examination. Neuromyotonia (24) and higher spontaneous activity (14) were seen on the EMG. Six cases of insomnia, two cases of absentee deep sleep, two instances of high-frequency beta activity, one point of REM behavior disorder, and one case of periodic leg movements were found in the polysomnography results of twelve patients.

Neuropsychological testing showed slight temporal and left frontal involvement. No cancers were identified during the workup. Each patient received steroids. Ten neuropathic pain patients had complete neurologic remission at follow-up.

Conclusion:

This work has advanced the understanding of Morvan syndrome linked to CASPR2. It is critical for greater awareness and early detection because immunotherapy may be able to treat it.

Recommendation:

Regularly get evaluated for the majority of common investigations, including brain MRIs, EEGs, PET scans, and CSF analyses. Individuals who should undergo regular evaluations are those presenting characteristic symptoms such as spastic speech, dysphagia, behavioral abnormalities, seizures, and neuromuscular issues.