Dr. Venumadhav, Dr.suma Balan(Paed Rheumato) Dr. C. Jayakumar
Department of Pediatrics, Amrita Institute of Medical Sciences
Kochi
Kerala
Fifteen year-old female child presented with sudden onset of Rt sided weakness, progressive in nature , involving Rt side of the face, UL and LL with slurring of speech& deviation of angle of mouth.
She had history of fatiguability past 3 months with recurrent short febrile illness. She also complaints of polyarthralgia predominantly involving knee and small joints, which was associated with early morning stiffness
She is developmentally normal and immunised for age.Parents noticed decreased academic performance for the past 3 months.
Examination
She was conscious and oriented .
Cranial nerve examination revealed Rt sided facial weakness with slurring of speech ,
No eye muscle or palatal muscle weakness observed.
Motor system examination revealed Rt sided hemiplegia.
Labs Hypo chromic micro cytic aneamia, leukopenia with lymphopenia. (TC-3800, N72,L17 Hb-10.2, Plt-372) with CRP(17.8) and Elevated ESR(107).
Above pictures showing child with UMN FN palsy.
Differential Diagnosis
Rt sided Henri plegia
Causes
Trauma
ICSOL
Bleed
Thrombosis
Cardiac causes
Infective Endocarditis
Hematological Hemoglobinopathies-Sickle felled disease
Infections Meningitis-Bacterial/TB
SLE-Vaculitis, Antiphospho lipid antibody syndrome
Moyà Moyà disease
Migraine
MRI Brain with Angiography done, showed acute infarcts in the left posterior limb of the internal capsule and right thalamus with no hemorhagic transfusion.
MR Angiogram of neck and intracranial vessels showed irregular focal narrowing in the distal branches of bilateral MCA and PCA, suggestive of vaculitus.
HPLC done showed no abnormal Hb peak and Thrombosis panel were normal.
ECG, ECHO and Doppler carotids done were normal.
ANA workup done showed 4 +, homogenous positive.
ANA blot was positive with dsDNA, Sm,RNA, Nucleosome.
RF factor done was negative.
DCT is weakly positive.
APS workup done was positive.
Physiotherapy was given daily. Based on this ,diagnosis of SLE with predominantly neuropsychiatry manifestation was considered
Treatment and outcome
She was started on Ecosprin(75mg) and Atorvastatin (10mg). After rheumatology consultation, she was commenced on Pulse methyl prednisolone( weaned off to oral steroids)
Due to CNS manifestation, she was given 2 doses of Rituximab(1gm/day).
She was added on MMF(Steroid sparing agent ) and prophylactic dose of septran prior to discharge.
Due to Dysarthira and Facial nerve palsy, she was kept on NG tube, slowly weaned to liquid, semi solid and solid foods over 2-3 days. Physiotherapy was continued at home.
DISCUSSION:
• Neuropsychiatric Systemic Lupus Erythematosus (NPSLE) presents a significant challenge due to its varying prevalence (14-80%) and diverse manifestations, ranging from severe neurological symptoms like seizures and psychosis to subtle cognitive impairments
The manifestations of NPSLE stem from different mechanisms such as antibodies, vasculitis, thrombosis, hemorrhages, and cytokine-mediated damage.
Diagnosis of NPSLE relies on a combination of immunoserological tests, functional and anatomical neuroimaging, and specific criteria due to the lack of a single diagnostic tool .
Treatment strategies for NPSLE include symptomatic, immunosuppressive, and anticoagulant therapies tailored to individual patient needs
Take Home message
Clinicians should maintain a high index of suspicion, especially in cases of sudden-onset neurological deficits in children, and promptly initiate diagnostic evaluation, including neuroimaging. Neuropsychiatric Systemic Lupus Erythematosus (NPSLE) presents a significant challenge due to its diverse neurological manifestations. Diagnosis requires a comprehensive approach including clinical evaluation, immunoserological tests, and neuroimaging