by Antonella Macerollo
An interesting interactive case has been published in eBrain this month. It has been written by the Neurology and Rheumatology Teams from Utrecht. Please, find below the abstract.
To see the full case, please log-in via the EAN-website, and once you are on the eBrain page, go to “virtual patient cases”:
A 69-year old lady with severe swallowing and speech problems: bulbar ALS?
Authors: A.M. Algra, Resident in Neurology, Dpt. of Neurology, A. van der Gijp, Radiologist / Fellow Neuroradiology, Dpt. of Radiology, C.J.M. Frijns, Neurologist, Dpt. of Neurology, J. Tekstra, Rheumatologist, Dpt. of Rheumatology, UMC Utrecht
A 69-year-old patient was referred to our neurology outpatient clinic because of rapidly progressing swallowing and speech difficulties, under the presumed diagnosis of ‘bulbar ALS’. Approximately a year before she had experienced ‘ghastly’ pains in her neck, after which she developed sudden deafness in both ears. The cardiologist found a high-grade AV-block, for which she received a pacemaker. We saw a cachectic patient (weight: 45 kilograms) with a PEG tube. At neurological examination we noted multiple cranial nerve palsies. Additional blood testing showed an elevated CRP and BSE, a weakly positive ANA, a positive MPO-ANCA, a mild proteinuria and a modest pleiocytosis in the cerebrospinal fluid without any malignant cells. A MRI of the neck and brain revealed pathological staining in the nasopharyngeal region of both internal carotid arteries and a thickened dura at the left base of the skull. A biopsy of the nasopharynx showed a lymphocytic infiltrate without any evidence for a malignancy, vasculitis or a granulomatous condition. We diagnosed our patient with a ‘MPO-ANCA associated’ vasculitis with systemic involvement of the lungs, heart, eyes, dura and possibly also kidneys. We treated her with intravenous methylprednisolone for three days, which immediately improved her swallowing and speech. Subsequently, our rheumatologist started her on a remission-induction treatment, consisting of prednisone 1mg/kg (with a reduction schedule) and six intravenous doses of cyclophosphamide. We prescribed prednisone 30mg/day and added two intravenous doses of rituximab (twice 1000mg, with a two-week interval) and azathioprine 100mg/day. Currently, our patient has made almost a full recovery; her latest MPO-ANCA levels are low (4.3 IU/ml). We continue follow-up in a multidisciplinary setting (neurologist, rheumatologist and ophthalmologist).
Please also visit the article about the Netherlands “country of the month” for June.