A rare case of Balo concentric sclerosis showing unusual clinical improvement and response with oral prednisolone
A 26-year-old male reported in August 2009, with complaints of acute-onset of loss of speech, deviation of face toward the right, nasal regurgitation of fluids, and altered behavior in the form of irrelevant and persistent smiling or crying, for the last 13 days. A day before, he had a few episodes of vomiting. There was no history of fever, headache, blurring of vision, diplopia, tinnitus, vertigo, seizures, unconsciousness, weakness of limbs, or bladder bowel involvement. There was no previous history of preceding infection or vaccination few days or weeks prior to the onset of neurological symptoms.\nDuring the examination, the patient was conscious, oriented with stable vitals, but aphasic (transient, motor), and left upper motor neuron type facial nerve palsy with ninth and tenth cranial nerve paralysis, in the form of an absent Gag reflex. It was due to the acute demyelinating phase of the disease and was consistent.\nNeck rigidity and Kernig's sign were absent; bilateral fundii, pupillary reflex, visual acuity, and color vision were normal with no ophthalmoparesis. The eighth, eleventh, and twelfth cranial nerves were also normal. The sensory and motor system and deep tendon reflexes were normal, the plantars were bilaterally downgoing, the Romberg sign was negative, and coordination was normal. There was no history of diabetes, hypertension, palpitation, breathlessness, photosensitivity, or joint pains in in past. No relevant family history was present.\nBlood biochemistry revealed normal blood count, and normal renal and liver function. Human immunodeficiency virus (HIV), anti-nuclear antibody, anti-dsDNA, rheumatoid factor, and anti-phospholipid antibody were negative. Differential diagnosis included confluent acute demyelinating encephalomyelitis (ADEM) and confluent lesions of MS.\nCerebrospinal Fluid (CSF) analysis showed a total white blood cell (WBC) count of 06 cells (all lymphocytes), sugar 36 mg%, protein 97.5 mg%, and adenosine deaminase 7.2 IU. It was negative for oligoclonal bands or IgG. T2-weighted MRI sequences [] and fluid-attenuated inversion recovery (FLAIR) sequence images showed bilateral asymmetric hyperintense confluent lesions in the periventricular and centrum semiovale location, suggestive of a demyelinating pathology []. Axial T2WI and gadolinium-enhanced scans showed characteristic concentric and laminated lesions [Figures. and ]. The lesions did not show any enhancement []. Magnetic resonance spectroscopy obtained as a single voxel image at 135 ms, at the time of presentation, showed a non-specific choline peak, suggestive of a demyelinating pathology [].\nThe patient was put on oral prednisolone based on clinical suspicion of an acute demyelinating pathology. The decision to continue with this was based on the dramatic response and clinical improvement of the patient. We started oral prednisolone 1 mg/kg for four weeks, and then tapered it over six weeks. The patient's speech, swallowing, and facial deviation returned to almost normal during the eight days of hospital stay. The patient was normal at the three-, six-, and twelve-month follow-up, with no relapse. Magnetic resonance imaging after 12 months showed reduction in size of the demyelinating plaques. On follow-up scans there was no evidence of dissemination of the disease in time or space. There were no new enhancing lesions, and no TI black holes were noted.