02511caa a2200253 4500 210629s9999 xx 000 0 und d 2377-2484 Appas Sahaa Pantothenate kinase-associated neurodegeneration (PKAN)–a rare clinicalentity Informa UK Limited 2016 Ali Yavar Jung National Institute for the Hearing Handicapped, Southern Regional Centre, Secundrabad, India Lecturer (Audiologyand Speech Therapy), Deparment of ENT, Swami Rama Himalayan University, Dehradun, Uttarakhand, India Reader in SpeechPathology, Departmentof Speech-Language Pathology, All India Institute of Speech and Hearing, Mysuru, Karnataka, India simpleshabnam@gmail.com 90–97 Volume 1 Issue 1 Pantothenate kinase-associated neurodegeneration (PKAN) is an autosomal recessive disordercaused by the absence or deficiency of PANK 2 gene located on the chromosome 20p13-p12.3.This is a motor disorder of extrapyramidal type with dystonia, rigidity, involuntary movements(choreoathetosis or tremor) and other pyramidal signs. We present the case of a patient, 13-year-old boy with PKAN who reported to our institute. The assessment revealed dystonia, dys-phagia, oro-motor weakness, increased tone in facial and cervical region, dysarthria, poor speechintelligibility and mild intellectual deficit. After a detailed assessment, the patient was recom-mended speech therapy, swallowing management and occupational therapy. He attended twosessions of EMG biofeedback therapy for the reduction of tone and 26 sessions of speech ther-apy. The EMG biofeedback therapy, relaxation exercises and massaging were helpful in reducingthe tonicity of the muscles and isotonic and isomeric exercises carried out during speech therapyimproved the oro-motor mobility and strength. He was also able to phonate the vowels withlesser effort. There was an improvement in the oral preparatory and oral transport phase of swal-low with no aspiration reported. Thus, it can be concluded that speech and swallowing manage-ment on a long-term basis is essential for patients with PKAN. Hallervorden–Spatz disease;pantothenate kinase-associated neurodegenera-tion; dysarthria; dysphagia Srushti Shabnamband Swapna, N ACTA OTO-LARYNGOLOGICA CASE REPORTS https://doi.org/10.1080/23772484.2016.1247649 FJAS FJAS 17719 17719