恶性非霍奇金淋巴瘤伴Garcin综合征(法国)

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Introduction. R Garcin described progressive unilateral cranialnerve palsy in 1926. Garcin syndrome is characterized by progressive involvement of the cranial nerves culminating intotal unilateral paralysis of all cranial nerves. Carcinoma of the skull base or ENT regions is the most common etiology.Case report. A 74- year- old man developed signs involving the left Vth (V2 and V3) cranial nerve then the VIth, VIIth and VIIIth cranial nerves and finally the IXth and Xth. MRI showed involvement of these cranial nerves with gadolinium uptake and involvement of the pons at the terminal phase. Careful ENT explorations failed to reveal a cause. The lymphocyte count was elevated in the cerebrospinal fluid. The patient died one year after diagnosis and the general autopsy was normal. The neuropathological studies led to the post- mortem diagnosis of type B non- Hodgkin lymphoma. Conclusion. In patients with Garcin syndrome, lymphoma is a possible diagnosis when carcinoma of the ENT regions or of the skull bases are not present. Introduction. Garcin syndrome is characterized by progressive involvement of the cranial nerves culminating intotal unilateral paralysis of all cranial nerves. Carcinoma of the skull base or ENT regions is the most common etiology. Case report. A 74- year- old man developed signs involving the left Vth (V2 and V3) cranial nerve then the VIth, VIIth and VIIIth cranial nerves and finally the IXth and Xth. MRI showed involvement of these cranial nerves with gadolinium uptake and involvement of the pons The patient died one year after diagnosis and the general autopsy was normal. The neuropathological studies led to the post-mortem diagnosis of type B with Hodgkin lymphoma. Conclusion. In patients with Garcin syndrome, lymphoma is a possible diagnosis when carcinoma of the ENT regions or of the skull bases are not present.
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