This chapter talks about an 83-year-old man with a 3-4-year history of progressive speech difficulty. Past medical history was remarkable for hypertension and ischemic heart disease. Psychiatric history was unremarkable. General neurological exam was remarkable for bilateral cogwheeling in the upper extremities, rigid posture, and bilateral decreased arm swing. Magnetic Resonance Imaging (MRI) of the brain showed diffuse atrophy. Single-Photon Emission Computed Tomography (SPECT) showed decreased perfusion in the left temporo-parietal region. The findings suggested a diagnosis of progressive non-fluent aphasia (PNFA). The possibility of corticobasal syndrome (CBS) was also raised. A variety of neuropathological changes have been associated with PNFA. The most common are non-Alzheimer tauopathies. Alzheimer pathology has also being identified in PNFA, with some reports showing these in up to 30% of cases. In these patients, the distribution of AD pathology may be unusual, showing a frontotemporal pattern.

This chapter talks about a 54-year-old right-handed man who was brought to medical attention by his daughter because of progressive speech difficulty over the last 2 years. The patient was clinically diagnosed with fronto-temporal dementia with non-fluent progressive aphasia as well as behavioral symptoms. Sensory and motor nerve conduction studies were normal. EMG needle electromyography showed mixed denervation pattern in the right FDI and left biceps with 2_ fasciculation potentials, positive sharp waves, and fibrillations. Motor units were polyphasic with increase in sharp waves. External examination of the formalin-fixed brain showed no obvious cerebral atrophy or no focal lesions. The base of the brain was unremarkable apart from mild patchy atherosclerosis. Serial coronal sections through the cerebral hemispheres showed a normal ventricular system and deep gray structures. Sections of the brainstem and cerebellum were also unremarkable apart from mild loss of pigmentation of substantia nigra.