Sheena Varughese1, Roy Abraham Kallivayalil1, Reji Thomas2. 1. Dept. of Psychiatry, Pushpagiri Institute of Medical Sciences and Research Centre, Thiruvalla, Kerala, India. 2. Dept. of Neurology, Pushpagiri Institute of Medical Sciences and Research Centre, Thiruvalla, Kerala, India.
Dear Sir,Multiple Sclerosis (MS) is a chronic inflammatory, demyelinating disease of the central
nervous system. It usually presents with limb weakness, visual disturbances, ataxia, bladder
and bowel dysfunctions, and sensory deficits.
Only rarely have psychiatric symptoms been reported as an initial manifestation of MS.
Here, we report an 18-year-old girl who presented with behavioral changes suggestive of
possible emotionally unstable personality traits associated with mixed affective state 18
months prior to the development of neurological symptoms of MS.
Case Description
Miss X, a temperamentally easy child, presented with gradual onset of behavioral changes
of six months duration. She had a decline in academic performance, excessive and
inappropriate interactions with boys, irritability, and two deliberate self-harm (DSH)
attempts. Her parents were separated for the past five years, and she was staying with her
mother. There was no significant medical, psychiatric, or family history. She reported no
first-rank symptoms, deterioration in personal hygiene or biological functions, or other
neurological or systemic symptoms.On examination, she was argumentative, irritable, and less concerned about the DSH
attempt and the consultation. No obvious cognitive or neurological deficits were noted.
Differential diagnoses of Emotionally Unstable Personality Disorder and mixed affective
episode were considered and treatment plans were discussed. However, she was lost to
follow-up, and no treatment was received.Excessive and inappropriate interactions with boys and irritability subsequently
persisted. The mother had to relocate near their college as the management was reluctant
to accommodate her in the hostel. After one year of the psychiatric consultation, she
developed acute onset right-sided weakness and episodes of transient blurring of vision.
In addition, there was a weakness of the right upper and lower limbs (Medical research
council grade 3) with positive Lhermitte’s sign. Mental status examination revealed
increased talk, euphoric mood, and intact cognitive functions.MRI brain (T2 and FLAIR [fluid attenuated inversion recovery]) revealed ovoid hyper
intense lesions in the periventricular and subcortical white matter, perpendicular to the
body of lateral ventricles (Dawson’s fingers, Figure 1), and irregularity of ependymal stripe on
the undersurface of the corpus callosum. Serum testing for antinuclear antibodies,
antibodies against double-stranded DNA, lupus anticoagulant, vitamin B12 levels, and
thyroid antibodies, among other tests, were negative.
Figure 1.
Cranial MRI Showing Sagittal T2-Weighted FLAIR Views of the Demyelinating Lesions
in the Juxtacortical Regions
CSF study revealed oligoclonal bands with elevated IgG index. Probable MS was diagnosed
based on the revised McDonald criteria.
Inj. Methylprednisolone 1 gm daily was given for five days, followed by glatiramer
acetate 20 mg per day, sodium valproate 1000 mg in divided dose, and psychotherapy
sessions. Her neurological and psychiatric symptoms improved remarkably following the
treatment of MS and the treatment with a mood stabilizer, and she is currently on regular
follow-up.
Discussion
This case report emphasizes that though rare, mixed affective symptoms and possible
emotionally unstable personality traits can be the initial manifestation of MS. The
psychiatric aspects of the MS have gained attention since the last century, wherein
intellectual and emotional disorders were noted to be frequent accompaniments during the
course of the illness, with psychotic disorders observed infrequently.In this case illustration, mixed affective symptoms, possible emotionally unstable
personality traits, and academic decline were the initial manifestations, and more typical
features of MS developed much later only. The psychiatric manifestations of MS can be
grouped into mood and cognitive disorders. Depression, bipolar disease, and pseudobulbar
affect are the common mood disorders, and irritability, apathy, disinhibition, and
euphoria are the other symptoms. The etiology of psychiatric manifestations is not fully understood.
The evidences suggest that psychiatric symptoms in MS are usually associated with
higher lesion load, specifically in the temporal periventricular area, and also with the
presence of cortical lesions. Thus, damage to the white matter, leading to disconnection
of the cortical areas and the basal ganglia, could be the underlying basis of the
psychiatric symptoms.
This patient had multiple periventricular and subcortical lesions, including in the
frontal and temporal regions. The orbitofrontal cortex is considered to be responsible for
socially appropriate behavior and empathy, that is, impulsivity, liability, and
personality changes. Disruption of the anterior cingulate cortex-orbitofrontal brain
circuitry is thought to explain the above symptoms in this patient.Cognitive deficits, common in MS, often manifested early and occasionally before the
onset of physical symptoms. Information processing, working memory and attention are
affected commonly.
Cognitive deficits may not be clinically observable early in the course, and the
“functional reorganization” could explain this. This is the brain’s compensatory mechanism
wherein the brain’s connectivity is altered to limit the expression of pathology. This
would partly explain the disparity between the observed lesions and the intact cognitive
functions in our patient.
Conclusion
Our case illustrates that although rare, mixed affective symptoms and possible
emotionally unstable personality traits can be a forerunner of other MS symptoms. Hence,
MS should be considered in the differential diagnosis of young patients with mood symptoms
and abnormal personality traits.
Authors: Chris H Polman; Stephen C Reingold; Brenda Banwell; Michel Clanet; Jeffrey A Cohen; Massimo Filippi; Kazuo Fujihara; Eva Havrdova; Michael Hutchinson; Ludwig Kappos; Fred D Lublin; Xavier Montalban; Paul O'Connor; Magnhild Sandberg-Wollheim; Alan J Thompson; Emmanuelle Waubant; Brian Weinshenker; Jerry S Wolinsky Journal: Ann Neurol Date: 2011-02 Impact factor: 10.422