Description

Balo’s concentric sclerosis is a rare inflammatory-demyelinating fulminant disease of the Central Nervous System (CNS). Although it is seen in the literature that the diagnosis is made postmortem histopathologically, antemortem diagnosis can be made with Magnetic Resonance Imaging (MRI). MRI lesions typically consist of rings of varying intensity.In this case we present, we present our patient who was diagnosed six months ago and was treated with high-dose corticosteroids because of a lamellar lesion in the left frontal region, but then presented with a new lesion in the right frontoparietal region. When the literature is searched, it is seen that the case of bilateral Balo's concentric sclerosis is rare.

Central Nervous System

Balo’s concentric sclerosis is a rare inflammatory-demyelinating fulminant disease of the Central Nervous System (CNS). Altough the first case was published by Otto Marburg in 1906, but it was defined as ‘’leukoencephalitis-periaxialis-concentric’’ by Hungarian neuropathologist Jozsef Balo in 1928. It is characterized by large and concentric lesions in the central nervous system. Although it is seen in the literature that the diagnosis is made postmortem histopathologically, antemortem diagnosis can be made with Magnetic Resonance Imaging (MRI). MRI lesions typically consist of rings of varying intensity. The lamellar pattern of the lesions reflects areas of demyelination and preserved myelin. Although the concentric ring appearance is not specific to Balo's concentric sclerosis; Concentric lesions have also been reported in patients with neuromyelitis optica (NMO), progressive multifocal leukoencephalopathy, hepatitis C, and human herpes virus 6 (HHV-6). Symptoms of the disease can usually occur in the form of speech disorder, limb weakness, gait disturbance, seizures, and behavioral changes.

   In this case we present, we present our patient who was diagnosed six months ago and was treated with high-dose corticosteroids because of a lamellar lesion in the left frontal region, but then presented with a new lesion in the right front oparietal region. When the literature is searched, it is seen that the case of bilateral Balo's concentric sclerosis is rare.

Case: A 52-year-old male patient was admitted to our outpatient clinic with complaints of sudden onset of forgetfulness, inability to remember words while speaking, slippage at the corner of the mouth, numbness in the left arm and leg, and loss of strength. The patient was examined in an external center, with the diagnosis of Balo's concentric sclerosis, he gave pulse steroid therapy for ten days. He did not see any benefit and applied to us because of his increasing symptoms. The patient, who had no known disease, medication, trauma, or history of febrile illness, was admitted to our neurology service for further examination and treatment. In his physical examination, temperature was 36.6 °C, blood pressure was 110/70 mmHg, heart rate was 107/minute, respiration was 18/minute, and oxygen saturation was 96. In his neurological examination, he was conscious, oriented and cooperative. Pupils were isochoric, light reflex was taken in both eyes, eye movements were free in all directions. His speech was minimally dysarthric, he had difficulty in finding words, and there was effacement in the left nasolabial groove. In the motor examination, the left upper and lower extremities were evaluated as plegic. Sensory examination was normal, cerebellar examination was skillful. Deep Tendon Reflexes (DTR) were hyperactive in both upper and lower extremities on the left, and plantar response was flexor.There was no sign of meningeal irritation. The Montreal Cognitive Assessment Test (Moca) was evaluated as 18 points.

Brain Tomography

    Routine blood tests, biochemistry, whole blood, vitamin b12 tests, thyroid function tests, HbA1c, erythrocyte sedimentation rate, serum electrophoresis, autoantibody screening (Antinuclear Antibody, anti-SSA, anti-SSB), antithyroid antibodies, syphilis serology (fluorescent treponemal antibody) ), Schirmer's test was normal. ANCA- immunoblot autoimmune panel- ANTI-DSDNA-RF-C3-C4-ACE-Anti-cardiolipin, NMO-Kappa-Lamda was normal. Elisa tests (HIV, Hepatitis A, Hepatitis B, Hepatitis C,  rubella, rubeola) were negative. Complete urinalysis was within normal limits. When viral meningitis factors were investigated, no factor was found. Brucella tests came back negative. Serum acetylcholine receptor antibody was negative.

Brain tomography (CT) dated 19.05.2021 in another center, it was reported that a wide hypo dense lesion area in the cortical subcortical part in the frontal left, compression on the left lateral ventricular frontal horn, and a minimal shift to the right in the midline formations were observed.

Concentric sclerosis of the Balo’s is rare, and the average age of onset appears to be thirty-four in the literature. It appears to affect women twice as often as men, with the most cases in Southern Han Chinese, Philippine and Taiwanese. Headache, cognitive impairment, speech and gait disturbances, loss of strength in the extremities, and seizures are the most common reasons for patients to apply. Balo's lesions may be confused with glial tumors and primary central nervous system lymphoma. While the diagnosis is made postmortem histopathologically before radiological imaging develops, antemortem diagnosis can be made due to the progression of MRI. On MRI, lesions are seen as isointense and hypointense rings. Although the concentric ring appearance is not specific to Balo's concentric sclerosis; concentric lesions have also been reported in patients with neuromyelitis optica (NMO), progressive multifocal leuko encephalopathy, hepatitis C, and human herpes virus 6 (HHV-6).