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BALÓ-LIKE LESION ASSOCIATED WITH PSORIASIS AND CHRONIC AUTOIMMUNE THYROIDITIS
Corina Roman-Filip1, Aurelian Ungureanu2, Ileana PrÄƒvariu3
Variants of multiple sclerosis are seldom encountered in clinical practice, sometimes with reserved prognosis or possibly serious disability. These pathologies are characterized by atypical demyelinating lesions such as Baló-type lesions or tumefactive lesions. The mechanism behind these lesions still remains a debate, since genetic and immune factors are incriminated. We present a case of multiple sclerosis variant with remarkably good outcome in association with autoimmune thyroiditis and psoriasis. This concomitant process questions the possibility of shared immune pathogenesis regarding the activation of T helper 17 cells lineage and mitochondrial oxidative stress. The imagistic appearance of the found lesions raises discussions on a possible radiologic diagnosis.
Keywords: Baló-type lesions; tumefactive lesions; psoriasis; autoimmune thyroiditis; T helper 17 cells;
Pathologist József Baló described a particular form of demyelinating disease, leukoencephalitis periaxialis concentrica, classically named Baló’s concentric sclerosis (BCS). Nowadays, this is defined as a variant of multiple sclerosis. The intensive use of magnetic resonance showed an increasing number of different types of demyelinating lesions. Some of these are specific, but a large variety is under debate regarding the classification. Tumefactive demyelinating lesions (pseudotumoral) can sometimes present a degree of concentricity and can be easily mistaken for a genuine Baló lesion, or at least a Baló-like demyelinating lesion. Historically, the variants of multiple sclerosis were regarded as serious disabling inflammatory damages of the central nervous system, but recent works have demonstrated that the course of the disease may be more variable, at least regarding BCS. Imagistic studies can lead to a better appreciation on the prognosis of BCS and its association with other types of demyelinating lesions . The pathological mechanism behind it still remains a debate, although one can find similarities with multiple sclerosis (MS) and even overlapping lesions of these conditions.
We present the case of a 40-year-old woman admitted for mild incoordination of the left arm and speech impairment. The patient’s medical history is positive for psoriasis (since 2002) and autoimmune thyroiditis under treatment with levothyroxine 50 ug/day (since 2010). Magnetic resonance imaging (MRI) studies revealed FLAIR and T2 weighted inhomogeneous hyperintense lesions with concentric enhanced and non-enhanced lesions on T1 with gadolinium contrast (fig 1ab). The lesion was characterized as atypical demyelinating with 22.5/21.6 mm in size, with late concentric enhancement and without mass effect. Additionally, two demyelinating periventricular enhancing lesions were found (fig 1def). A biochemistry panel, antinuclear antibodies, anti-ds DNA antibodies, ANCA antibodies, anti Ro antibodies and anti-Borrelia antibodies were negative. Slight pleocytosis (16 cells/mm3 with 75% monocytes) was detected in the cerebrospinal fluid together with present oligoclonal bands and normal proteins. Serum myelin oligodendrocyte glycoprotein antibodies, myelin basic protein antibodies, IgG anti-aquaporin 4 antibodies were negative. Moreover, a high serum titre of anti-thyroperoxidase antibodies (60.73 IU/mL – normal<5.6 IU/mL) was present with normal TSH, FT3 and FT4 under treatment with levothyroxine. The clinical and imagistic assessments suggested Baló-like demyelinating lesion associated with psoriasis vulgaris and autoimmune endocrinopathy. After 5 days of intravenous methylprednisolone, one gram per day, we observed significant improvement. A differential diagnosis with MS, acute disseminated encephalomyelitis, lymphoma and central nervous system tumour was made, but all were ruled out by the clinical and imagistic studies. Tumefactive lesion is still a very highly probable radiologic diagnosis. The dimensions must be larger than 2 cm in diameter, with mass effect and open ring contrast enhancement, with serious disability, which was excluded. Six months later, MRI was significantly improved (fig 2) with sustained clinical remission and radiologic improvement. The biochemistry reassessment found high liver enzymes and the patient was evaluated for viral and autoimmune hepatitis (negative anti-mitochondrial antibodies, anti-LKM1 and anti-smooth muscle fibre antibodies, negative virology). One year later, the patient presents no neurological impairment without any other immunomodulation or immunosuppressive treatment assigned.
The association of the pathologies described may seem incidental. However, strong research evidence shows the implication of Thelper17 cells (Th17) and Interleukin 17 (Il17) in the autoimmune pathways of MS, autoimmune endocrinopathy and psoriasis [2,3]. BCS type lesions and MS lesions may both be present simultaneously in the same patient, and Baló-like lesions may change over time into the classic appearance of MS lesions . The lesions are characteristic, with rings of demyelination, surrounded by partial demyelinated regions, reflecting the concentricity within the lesion. The lesion type is classified as MS pattern III with oligodendrocyte loss, microglial activation and loss of myelin-associated glycoprotein . Studies of 7 Tesla MRI support the microvascular pathology associated to inflammation, which seems to be consistent with pattern III lesions . These studies are sustained by identifying Notch 3 mutation in a patient with BCS phenotype and a family history of Notch 3 mutation carriers and CADASIL. Mitochondrial respiratory chain disturbance and the expression of some molecules probably tend to precondition hypoxic tissue to inflammation, such as mitochondrial heat shock protein 70 . Furthermore, new cellular biology studies of cancer found that hsp70 can mediate the Th17 differentiation . We consider that the simultaneity with the autoimmune endocrinopathy and psoriasis may be more than incidental and raises the hypothesis of probable linkage of the proinflammatory and autoimmune role of Th17 cells lineage with mitochondrial oxidative stress.
Compliance with Ethical Standards
Conflict of Interest: The authors declare that they have no conflict of interest
Informed consent was obtained from all individual participants included in the study
Fig. 1 a. T1 gadolinium sequence showing a frontal demyelinating lesion with concentric enhancing rings (arrow); b. T2 weighted image with concentric rings of demyelination and myelinated regions (arrow); c. DWI sequence with diffusion restriction in the active lesion; d. T1 gadolinium enhancement of periventricular lesion (arrowhead); e. Enhancing periventricular lesion in the occipital lobe (arrowhead); f. Coronal T2 – small demyelinating lesion (arrowhead) with enhancement on T1 (not shown)
Fig. 2 a. T1 gadolinium sequence showing a significant improvement six months later (arrow); b. T2 weighted image showing the demyelinating lesion markedly decreased (arrow); c. DWI; d, e, f. Improvement of demyelinating lesions
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