Also known as:
Foster Kennedy's syndrome
OR (different disease, same name)
Spinal Bulbar Muscular Atropy (SBMA)
The first "Kennedy's syndrome" was named in 1911 for Foster Kennedy, who made the first extensive and thorough account of the condition. This syndrome is characterised by unilateral ipsilateral atrophy with contralateral papilloedema, central scotoma, and anosmia (absence
of the sense of smell) usually due to a frontal lobe tumor or a meningioma of the optic nerve. There may be headache,
dizziness, vertigo; occasionally, forceful vomiting, psychic changes (moria), memory loss.
The second syndrome to bear this name, a.k.a. Spinal Bulbar Muscular Atropy, was named for another Kennedy in 1968, and is characterized by slowly progressive muscle weakness associated with mild androgen insensitivity. Only males are affected.
Symptoms typically begin between the ages of
20 and 50 years, and begin with difficulty walking and a tendency to fall. After one to two decades of symptoms, most patients have difficulty climbing stairs. Typically, about one-third of patients require a
wheelchair after two decades of symptoms.
Most patients eventually show involvement of the bulbar muscles and have difficulty with speech articulation and swallowing. Severely affected patients are at risk for asphyxiation or aspiration pneumonia because of weakness of the bulbar musculature. This complication is the
only life-threatening problem in SBMA, and probably only becomes important for about 10% of elderly males. Therefore, the vast
majority of patients with SBMA have a normal life expectancy and do not die from direct complications of their