Barbara Tomik(1), Dariusz Adamek(2), Anna Lechwacka(1), Miroslawa Orlowiejska-Gillert(1), Maria Bala-Slodowska(1), Lidia Glodzik-Sobanska(1), Monika Kusiak(1), Andrzej Szczudlik(1)

ALS-Plus Syndrome. A Clinical and Neuropathological Case Study

1) Department of Neurology,
2) Department of Neuropathology, Jagiellonian University Medical College, Krakow

Abstract

ALS-Plus syndrome occurs rarely and usually presents typical ALS phenotype associated with dementia, Parkinsonism or both. We reported a case of sporadic, definite ALS with pseudobulbar palsy, emotional lability and selective cognitive deficit in the presence of frontal lobe dementia. Neuropsychological tests predominantly demonstrated perseveration and dynamic apraxia , CT and MRI scans showed widened subarachnoideal spaces in the frontal and temporal regions. The neuropathological findings confirmed ALS processes i.e. atrophy of motor nuclei in brainstem and anterior horns of cervical spinal cord and showed mild atrophy and status spongiosus in the frontal lobes. These findings suggest the co-occurrence of sporadic ALS and frontal lobe dementia: ALS- Plus syndrome.