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|Year : 2003 | Volume
| Issue : 4 | Page : 528-530
Concentric sclerosis: Imaging diagnosis and clinical analysis of 3 cases
Gu J, Wang R, Lin J, Fang S
Institute of Biomedical Engineering of the Huazhong University of Science and Technology, 1037 Luoyu Ave, Wuhan, 430074
Institute of Biomedical Engineering of the Huazhong University of Science and Technology, 1037 LuoYu Ave, Wuhan 430074
Baló’s Concentric sclerosis (BCS) is a rare demyelinating disease considered to be a variant of multiple sclerosis (MS). The typical magnetic resonance imaging (MRI) changes associated with BCS consist of concentric rings or onions’ cross-section on T1-weighted (T1W) images. Because MRI reveals pathological changes consistent with autopsy in the focus of BCS, it plays an important role in the before-death diagnosis of BCS. We report three cases of BCS diagnosed antemortem on the basis of the typical concentric rings pattern on MRI and on the basis of clinical findings and cerebrospinal fluid (CSF) examination. BCS often occurs in the prime of life, acutely or subacutely. Then come cerebral multifocal symptoms and signs. We find that BCS is not always an acute and irreversible pathological process as described in the past.
|How to cite this article:|
Gu J, Wang R, Lin J, Fang S. Concentric sclerosis: Imaging diagnosis and clinical analysis of 3 cases. Neurol India 2003;51:528-30
Baló's Concentric sclerosis (BCS) is a rare demyelinating disease considered to be a variant of multiple sclerosis (MS). The characteristic pathological findings are alternating rings of myelin preservation or remyelination and myelin loss, which appear as concentric pattern streaks alternating between light and shade, resembling trees' annual rings or onions' cross-section., Less than 60 cases have been reported in the literature. Our 2 case reports support the concept that BCS may be a self-limiting disease that is not always fatal and the MRI appearance of the chronic lesion may resemble that of a chronic MS plaque.,, Characteristic MRI findings may allow antemortem diagnosis of BCS when performed at the onset of the disease.,
A 35-year-old man was hospitalized because of acute onset of left hemiparesis. CT scan revealed low-density focuses in the right frontal, temporal and parietal lobe [Figure - 1]. Local treatment for cerebral infarction was not effective. Left hemiparesis began to aggravate. The patient received IV administered Dexamethasone (15 mg for 4 days) and 20% Mannitol (125ml q8h for 4 days). After 1 week, the patient showed mild clinical improvement. On examination there was upper motor neuron type facial paralysis on the left, power of the left upper and lower limbs MRC grade 4/6 and 3/6 respectively, and positive left Babinski sign. MRI T1WI revealed 3-5 concentric rings with diameters ranging from 2 to 4.5 cm, alternating between black and white (between low and equal signal) in the white matter of the above-mentioned lobe, resembling trees' annual rings [Figure - 2]. On T2WI, the above-mentioned rings' focus appeared as high signal, the diameters were slightly longer than on T1WI but the black-and-white zones turned unnoticeable.
Reinforced MRI scan revealed distinct incomplete rings reinforcement in the right temporal lobe. After 1 month, MRI scan revealed the following: the focus in the above-mentioned lobe. became smaller; and concentric rings were visible on T2W and T1W. CSF examination disclosed a normal routine and biochemical, negative oligoclonal bands. Dexamethasone (15 mg/day) was IV administered for 15 days, and the patient's condition improved within a few days. Three weeks later, the results of the patient's neurological examination revealed muscle power of the left upper and lower limbs recovered to MRC grade 5/6 and 4/6 respectively. During 3 years of follow-up, there were no further relapses. Half a year later, MRI revealed that the focus decreased remarkably (diameter: 1-2 cm). Final MRI, performed approximately 3 years after the first attack, showed that the concentric pattern had completely disappeared and that only a small, residual non-specific, gliotic, plaque-like lesion remained. No additional lesion was detected [Figure - 3].
A 38-year-old woman was hospitalized because of acute four limbs paralysis, apathy and epilepsy. Head CT revealed several circle-like low-density focuses in bilateral cerebral hemispheres. Local treatment for brain abscess was not effective. A neurological examination revealed drowsiness, upper motor neuron type facial and lingua paralysis on the left, four limbs paralysis, and positive bilateral Babinski sign. CSF examination revealed a normal biochemical, positive oligoclonal bands and leucocyte 0.02×109/L (range,0-5×106/L). MRI T1WI revealed several concentric pattern focuses alternating between low and equal signal in bilateral frontal, temporal and parietal lobe, resembling trees' annual rings or onions' cross-section. The proposed diagnosis was BCS and corticosteroids were administered. The patient showed left hemiparesis. After 1 month the patient died of lung infection.
A 40-year-old man presented with headache, euphoria, epilepsy and acute left hemiparesis. Head CT revealed multiple lesions. A neurological examination during hospitalization indicated cognitive dysfunction, positive grasp reflex, muscular power, and normal tension of all limbs. CSF examination disclosed a normal routine and biochemical, negative oligoclonal bands. MRI T1WI revealed a few concentric lesions located in the white matter of the left frontal and parietal lobe [Figure - 4]. The proposed diagnosis was BCS. The patient received high dose IV-administered Dexamethasone (15 mg/day for 15 days). After 1 month, the patient showed significant clinical improvement. CT revealed that the focus was remarkably diminished. During 1-and-a-half years of follow-up, there were no further relapses. No additional lesion was detected.
The group of 3 cases observed were diagnosed as BCS, based on the characteristic concentric rings found on MRI and clinical findings. Kim Mo and Boloy's hold that the etiological factor of BCS is possibly the same as that of MS. Currently, the common viewpoint is that BCS is a variation of MS. Yao holds that, in its pathogenesis, the loss of oligodendrocytes plays an important role in the demyelination. The courses of Cases 1 and 3 were longer and the prognosis was good in the 3 cases, which is different from the Weinshenker BG reports while consistent with Boloy's findings. In all the 3 cases, the CT-proposed diagnosis was cerebral infarction, brain tumor, brain abscess, inflammatory granuloma, etc.; MRI, especially TWI, revealed concentric patterns alternating between low and equal signal in white matter, resembling onions' cross-section; it was even more distinct with Gd-DTPA.
In past diagnosis of BCS was mainly based on autopsy., Currently, besides the biopsy of brain, MRI is the main diagnostic means for BCS before death as MRI reveals pathological changes consistent with autopsy in the focus of BCS.,,, The combination of proton magnetic resonance spectroscopy (1H-MRS) and the typical MRI revelation suggested by Kim and Ercan Karaarslan may take the place of brain biopsy in the diagnosis of BCS when performed at the onset of the disease.
Early treatment with sufficient glucocorticoid can remit clinical symptoms. The 3 cases were all treated with Dexamethasone 15 mg for 10-15 days. The clinical symptoms of two cases got a relatively long period of remission. The patients did not experience any relapse for 1-and-a-half to 3 years. As to the prognosis of BCS, previous reports included an extremely dangerous state, short course, quick progress to death with a survival period ranging from several weeks to months. However, according to the cases diagnosed antemortem by biopsy of brain or MRI in recent years,,, although the onset of illness is acute and the patient's condition is serious, the clinical symptoms of patients remit partially or disappear completely after timely treatment, which is consistent with our Cases 1 and 3. This suggests that Balo's disease is not always an acute and irreversible pathological process as described in the past.
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