title: An autopsy case of frontotemporal dementia with severe dysarthria and motor neuron disease showing numerous basophilic inclusions.
abstract: We report a clinicopathological study of a patient suffering from frontotemporal dementia (FLD) with severe dysarthria and concomitant motor neuron disease (MND). The patient was a 52-year-old woman with almost simultaneous emergence of severe dysarthria and FTD. The severe dysarthria subsequently evolved into anterior opercular syndrome. Motor neuron signs then emerged, and the patient developed akinetic mutism approximately 2 years after the onset of the disease. The patient died of pneumonia after a 7-year clinical illness. Pathologically, severe and widespread degeneration in the frontal and temporal lobes, including the anterior opercular area, limbic system, basal ganglia, spinal cord and cerebellum, and frequent ubiquitin- and tau-negative basophilic inclusions were observed. The pyramidal tracts and anterior horns of the cervical cord also showed marked degeneration. Cases showing basophilic inclusions reported so far have been divided into two groups: early onset FTD and MND with basophilic inclusions. Our case presented clinicopathological features of both FTD and MND, which suggests that cases showing basophilic inclusions may constitute a clinicopathological entity of FTD/MND.
authors.affiliation: Department of Neurology, Showa University School of Medicine, Hatanodai 1-5-8, Shinagawaku, Tokyo 142-8666, Japan. k-ishihara@mvj.biglobe.ne.jp
authors.forename: Kenji
authors.initials: K
authors.lastname: Ishihara
authors.forename: Shigeo
authors.initials: S
authors.lastname: Araki
authors.forename: Nami
authors.initials: N
authors.lastname: Ihori
authors.forename: Jun-ichi
authors.initials: J
authors.lastname: Shiota
authors.forename: Mitsuru
authors.initials: M
authors.lastname: Kawamura
authors.forename: Imaharu
authors.initials: I
authors.lastname: Nakano
mesh_terms: D001344:Autopsy; D001921:Brain / Q000473:pathology*; D003704:Dementia / Q000150:complications* / Q000473:pathology / Q000503:physiopathology; D004401:Dysarthria / Q000209:etiology*; D017034:Epilepsy, Frontal Lobe / Q000209:etiology; D005260:Female; D006801:Humans; D002479:Inclusion Bodies / Q000473:pathology*; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000150:complications* / Q000473:pathology / Q000503:physiopathology; D009046:Motor Neurons / Q000473:pathology; D013116:Spinal Cord / Q000473:pathology; D013966:Thyroiditis / Q000150:complications