Systemic lupus erythematosus (SLE) is an autoimmune disease which has an evidence of multifactorial etiopathogenesis. Neuropsychiatric SLE (NPSLE) can have variable presentation ranging from nonspecific vague symptoms like headache to the most devastating CNS manifestations like limbic encephalitis (LE). Limbic encephalitis in SLE, remains poorly understood, with a prevalence ranging between 12% to 95%. Stroke, seizure disorder, acute confusional state, cognitive decline and neuropathies encompass the diverse features in NPSLE. It has been reported that 10% of lupus patients have a first or second degree relative with SLE. Here we describe, chronic psychiatric illness masquerading as bipolar disorder in a family secondary to familial SLE and limbic encephalitis.
A 26-year-old lady presented with insidious onset, progressive behavioral disturbances for 4 years, in the form of excessive irritability, decreased self-care, and inability to communicate and maintain attention and inability to perform all the activities of daily living. She also had episodes of mood elation and depression. She was diagnosed as bipolar disorder with 2 episodes of mania per year. She was on olanzapine 7.5 mg and lamotrigine 100 mg daily. She was bed bound for the previous 2 months. She was brought in an unconscious state.
Examination revealed Glasgow Coma Scale; E3M1V1, and the patient was stuporous. Her blood pressure was 80/60 mmHg, pulse rate was 110/min, temperature of 100 F. Her oral hygiene was poor, she had anasarca, sacral region showed Grade 4 bedsore. She was unable to comprehend verbally, and there was no speech output. Pupils were normal, with bilateral horizontal gaze palsy indicating pontine brain lesion. There were stereotypical movements of perioral region and both upper limbs which were arrhythmic, irregular, involving only distal upper limb in the form of flexion extension of the digits. She remained in an akinetic rigid state with minimal movements of upper limbs. Deep tendon reflexes were brisk and plantar's were extensors. Provisional diagnosis of delirium with septic shock secondary to bedsore on a background of chronic encephalitis was considered. Investigation is shown in Table 1.
MRI brain showed trident sign in pons [Figure 1], temporal atrophy [Figure 2], and claustrum atrophy [Figure 3]. She improved subsequently with care of bedsore and antibiotics. Antiepileptic drugs were initiated for involuntary movements typical of faciobrachial dystonic seizures. Steroids for systemic lupus erythematosus (SLE) encephalitis could not be initiated in view of bedsore positive for methicillin-resistant Staphylococcus aureus. She was discharged in a stable condition with improvement in her movements. On further interview with relatives, it was found that her mother and elder sister had behavioral disturbances and were also on antipsychotic medications. Serological markers asked for these relatives were positive for SLE.
SLE can mimic like limbic encephalitis (LE) thus making the diagnosis even more challenging. It has been reported that 20% of SLE patients develop a chronic mild psychotic disorder. SLE is found to be independently associated with bipolar disorder. Familial SLE has been described and it indicates a basis of genetic influences in its pathophysiology. It has been observed that familial SLE had a female preponderance, a concordance in the age of presentation and clinical features was observed and a role for HLA DR2 was found. Pontine lesion in MRI brain in our case was consistent with osmotic demyelination secondary to sepsis and hypotension while claustral and temporal atrophy clued to chronic limbic involvement of brain due to SLE. As most of the autoimmune diseases are amenable to treatment, hence early recognition remains the key.
This case throws light on the following aspects. SLE can masquerade as LE with bipolar disorder features. Misdiagnosis of neuropsychiatric systemic lupus erythematosus (NPSLE) can have catastrophic adverse events and inappropriate management. NPSLE can have a familial presentation. There is a need for the evaluation of reversible and systemic causes of psychiatric presentations.
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