Atypical motor neuron disease, progressive bulbar palsy: A case report

Abstract

Motor Neuron Disease (MND) is a rare illness that involves the progressive neurological deterioration of upper and lower motor neurons. This case report presents a 52-year-old female with atypical MND progression. Usually, for patients with this diagnosis life expectancy ranges from 2 to 5 years from the onset of symptoms with their health rapidly deteriorating. The first diagnosis of MND occurred in this patient’s medical history in 2016 with few symptoms that lingered for 3 years with no visible illness progression. In 2016 a 44-year-old woman was admitted to the hospital with progressive speech impairment. A head MRI revealed a cerebral aneurysm in the right median brain artery, it was clipped, but the symptoms worsened. Electroneuromyography (ENMG) concluded that the patient suffers from motor neuron disease. In 2019 she was admitted for assessment of disease progression. For 3 years she was treated with Riluzol. Objectively tongue atrophy and multiple fibrillations were present. Limb strength - 5 points with no alterations. For further assessment, ENMG was performed. It revealed spontaneous activity and active denervation in m. trapezius dexter. As well as chronic mild reinnervation lesions. For the next 3 years patient would come back for check-ups with no noticeable disease progression. In 2022 patient’s limb strength remains - 5 points. She has pronounced dysarthria, no posterior pharyngeal wall reflex and small fibrillary jerks on the sides of the tongue. The condition has been deteriorating for about a year. Her speech has become completely incoherent and she interacts via text messages. Swallowing solid food also became difficult. The patient was referred to an otoneurologist who diagnosed – Glossoplegia, bilateral N. hypoglossus lesion, partial bulbar phenomena. Another head MRI showed diffuse, symmetrical tongue atrophy with muscle degeneration. Atrophic mouth floor muscle, nasopharynx and oropharyngeal wall lesions. Today the patient’s endured 8 years with an MND diagnosis and, unfortunately, is immobile and requires full-time care. The discussion on MND is multidimensional. Research is important for effective strategies for diagnosis and treatment. MND presents a challenge to both patients and healthcare professionals. While illness etiology remains unknown, the impact on the patient’s life quality is undeniable. Current treatments focus on the relief of symptoms, as there is no cure for MND now.