Diagnosis:
A combination of clinical and MRI
Clinical:
- Age <65yo
- Parkinsonism: Rigidity, bradykinesia, falls
- Dopamine resistant
- Cortical features: Hand clumsiness, apraxia, language dysfunction, Mute,
MRI:
- Frontal atrophy, caudate atrophy, temporal atrophy,
Pathology:
- Gross: frontal atrophy, caudate atrophy, Nigral pallor,
- Superficial Spongiosis in frontal lobes, Purkinje cell loss
- Intraneuronal inclusions (cytoplasmic) in frontal lobes, see below
Immunohistochemsitry:
- Intraneuronal Inclusions (cytoplasmic & intranuclear) are neurofilament positive, ubiquitin positive.
- Inclusions are negative for alpha synuclein, tau, amyloid, prion protein