A Rare Syndrome Causing Neurogenic Dysphagia
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CLINICAL CONUNDRUM
A Rare Syndrome Causing Neurogenic Dysphagia Antonio Zito1 · Paola Bini2 · Massimiliano Todisco1,3 · Giuseppe Cosentino1,3 · Simone Mauramati4 · Matteo Paoletti5 · Vincenzo Marasco6 · Enrico Marchioni2 · Enrico Alfonsi3 Received: 30 June 2020 / Accepted: 27 October 2020 © The Author(s) 2020
History A 46-year-old right-handed man with a medical history of refractory stage IIIB, ALK-negative, anaplastic large-cell lymphoma came to our attention. He had previously been treated with several chemotherapy drugs, including brentuximab vedotin. Two weeks after completing salvage chemotherapy, the patient presented with progressive swallowing and speech difficulties. At this stage, he was admitted to our hospital for further diagnostic work-up and clinical management. Neurological examination showed severe dysarthria and bilateral weakness of facial, oromandibular and palatal voluntary movements. The patient could barely open his mouth and protrude his tongue on command. However, reflex movements (e.g., blink and gag reflexes) and activation of emotional and automatic motor responses (e.g., facial response to painful stimulus, laughing at jokes, mouth opening during yawning) were broadly spared. Blood tests showed severe lymphocytopenia (77/mm3). Brain magnetic resonance imaging displayed T1-hypointense, T2-hyperintense abnormalities of the frontal subcortical white matter, also involving the U-fibers and the frontal Antonio Zito and Paola Bini have contributed equally to the manuscript. * Massimiliano Todisco [email protected] 1
Department of Brain and Behavioral Sciences, University of Pavia, Pavia, Italy
2
Neuroncology and Neuroinflammation Unit, IRCCS Mondino Foundation, Pavia, Italy
3
Clinical Neurophysiology Unit, IRCCS Mondino Foundation, Via Mondino 2, 27100 Pavia, Italy
4
Department of Otorhinolaryngology, University of Pavia, IRCCS Policlinico San Matteo Foundation, Pavia, Italy
5
Neuroradiology Unit, IRCCS Mondino Foundation, Pavia, Italy
6
Università degli Studi di Milano, Milan, Italy
operculum bilaterally (mainly on the right side). No mass effect or contrast enhancement after gadolinium administration was evident (Fig. 1). On both T1- and T2-weighted sequences, the Milky Way sign was also observed (i.e., multiple point-like alterations surrounding the main lesions) (Fig. 1). Magnetic resonance spectroscopy (at intermediate echo time of 135 ms) of the right opercular lesion showed increased choline and decreased N-acetyl aspartate levels, and a lactate peak, indicating the presence of neuronal loss, necrosis and increased cell turnover. Cerebrospinal fluid (CSF) analysis showed a normal cell count, normal albumin quotient, and absence of oligoclonal banding, but the polymerase chain reaction was positive for JC virus DNA (390 copies/mL). To allow more in-depth exploration of the features of neurogenic dysphagia in this patient, he underwent an electrokinesiographic/electromyographic study of swallowing (EES), which was performed simultaneously with a fi
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