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Case Reports
. 2017 Apr 5:8:44.
doi: 10.4103/sni.sni_401_16. eCollection 2017.

Systemic sclerosis associated with colliquative necrosis in the cerebellum

Alessandro Ricci  1 , Hambra Di Vitantonio  2 , Danilo De Paulis  1 , Mattia Del Maestro  2 , Soheila Raysi Dehcordi  2 , Domenico Murrone  2 , Gino Coletti  3 , Giuseppe Calvisi  3 , Renato Juan Galzio  2
Affiliations
Case Reports

Systemic sclerosis associated with colliquative necrosis in the cerebellum

Alessandro Ricci et al. Surg Neurol Int. .

Abstract

Background: The scleroderma is a complex autoimmune collagen disorder that can affect many organs simultaneously, as it occurs in the systemic sclerosis (SS), or only the skin, as it occurs in the localized scleroderma (LS). The neurological presentation is extremely uncommon, and even more uncommon are the symptoms of the scleroderma in the cerebellum.

Case description: We report the case of a 56-year-old male with cerebellar lesions mimicking a brain abscess. After surgical excision, the histopathological diagnosis deposed for an ischemic necrosis caused by a vasculopathy. All the bacteriological and viral exams were negative, whereas the rheumatologic tests were compatible with the scleroderma pattern.

Conclusion: Up to now, the literature has described only 5 cases of scleroderma in the posterior cranial fossa. The authors report a case of SS causing colliquative necrosis in the cerebellum. Pathogenetic mechanisms, clinical aspects, and radiological features are discussed along with the pertinent literature.

Keywords: Cerebellum; colliquative necrosis; posterior cranial fossa; scleroderma.

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Conflict of interest statement

There are no conflicts of interest.

Figures

Figure 1
Figure 1
Preoperative magnetic resonance image in axial, coronal, and sagittal view showing a lesion with fluid collection, measuring 1.5 × 3.5 cm. localized in the right cerebellar hemisphere (a-c)
Figure 2
Figure 2
After suboccipital craniotomy, the intraoperative visualization showed a subcortical colliquative necrosis (a and b). The wall of the lesion was resected (c) and the fluid collection was completely free-hand evacuated (d)
Figure 3
Figure 3
Imaging in hematoxylin and eosin and original magnification ×20 and ×40 showed cerebellar necrosis associated with microparenchymal hemorrhage, leukocytoclastic vasculitis, and swollen endothelial cells (a and b). Immunohistochemistry imaging (CD 68 +) showed massive macrophages infiltration in the cerebellar parenchyma (c and d)
Figure 4
Figure 4
Postoperative magnetic resonance in axial, coronal, and sagittal view showing the complete resection of the lesion (a-c)
See this image and copyright information in PMC

References

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