Symptoms, diagnostic and differential diagnostics of an ENT rarity

A 62-year-old male with a SCC of the base of the tongue (pT2 pN0 cM0 R0) underwent transoral resection of the tumour, plastic reconstruction with a radial forearm flap, bilateral neck dissection and tracheostomy in March 2021. The initial postoperative recovery was uneventful. The patient was discharged from intensive care on the first postoperative day and early mobilisation could be started. As his swallowing function was satisfactory, an uncuffed tracheostomy tube was inserted and oral food intake could be started under the supervision of the speech and language therapists in the first few days after surgery.

Approximately one week after the surgery, a decrease in mobility was noted. The initially fully independently mobile patient needed full assistance to mobilise within days. His handwriting became illegible, his speech was progressively slurred and he developed a gaze-evoked nystagmus. No explanation for these events could be found in the initially performed investigations including CT and MRI scans. The neurologists diagnosed a progressive tetraataxia, most likely due to a paraneoplastic process. Due to the rarity of this syndrome in conjunction with SCC of the head and neck, we excluded the presence of a secondary tumour. In the liquor analysis, ARHGRP-antibodies, which are known to cause irreversible damage to the Purkinje cells, were found. A therapy with intravenous corticosteroids, immunoglobulins and plasmapheresis did not lead to an improvement or a stabilisation of the findings. An alternative reason for the presence of the antibodies could not be found. In conclusion, the patient suffered from a subacute paraneoplastic degeneration of the cerebellum due to ARHGRP-antibodies, which has so far not been described in the presence of a SCC.

Conflict of Interest

The author declares that there is no conflict of interest.

Publication History

Article published online:
24 May 2022