A case of amyotrophic lateral sclerosis with frontotemporal dementia manifested as naming and sentence comprehension disorder

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AbstractBackgroundApproximately half of the patients with motor neuron disease (MND) will develop frontotemporal dysfunction. Most of case reports showed a strong association between amyotrophic lateral sclerosis (ALS) and behavioral variant frontotemporal dementia (bvFTD) and mostly presented as FTD at the onset, while the relationship between ALS and primary progressive aphasia (PPA) has not been fully understood.MethodA patient suspected to be ALS‐PPA admitted to Beijing Tiantan Hospital, Capital Medical University in January 2020. We want to describe this case to summarize its clinical characteristics.ResultThe patient is 70 years old,female, illiteracy and with severe hearing impairment. She developed bulbar paralysis manifestations like hoarseness, dysarthria and dysphagia without obvious mental, character and behavior changes in the course of the disease.Both upper limbs tendon reflex was hyperactive and lower limbs tendon reflex was decreased. Sucking reflex existed. Neuropsychological test suggested dementia, impaired naming and sentence comprehension. Electromyography suggested neurogenic changes around the upper and lower extremities. Magnetic resonance imaging showed symmetrical anomaly signal of bilateral basal ganglia, where manifested as low metabolism on PETCT. Amyloid β (1‐42) was elevated, the total protein Tau was reduced and the phosphorylated tau protein was normal in cerebrospinal fluid.ConclusionALS‐PPA is a relatively rare disease. Sometimes maybe genetic and pathological examinations are required for diagnosis.