Dementia (cont.)
Secondary Dementias
Dementia may occur in patients who have other disorders that primarily affect
movement or other functions. These cases are often referred to as secondary
dementias. The relationship between these disorders and the primary dementias is
not always clear. For instance, people with advanced Parkinson's disease, which
is primarily a movement disorder, sometimes develop symptoms of dementia. Many
Parkinson's patients also have amyloid plaques and neurofibrillary tangles like
those found in AD. The two diseases may be linked in a yet-unknown way, or they
may simply coexist in some people. People with Parkinson's and associated
dementia sometimes show signs of Lewy body dementia or progressive supranuclear
palsy at autopsy, suggesting that these diseases may also overlap with
Parkinson's or that Parkinson's is sometimes misdiagnosed.
Other disorders that may include symptoms of dementia include multiple
sclerosis; presenile dementia with motor neuron disease, also called ALS
dementia; olivopontocerebellar atrophy (OPCA); Wilson's disease; and normal
pressure hydrocephalus (NPH).
Dementias in Children
While it is usually found in adults, dementia can also occur in children. For
example, infections and poisoning can lead to dementia in people of any age. In
addition, some disorders unique to children can cause dementia.
Niemann-Pick disease is a group of inherited disorders that affect metabolism
and are caused by specific genetic mutations. Patients with Niemann-Pick disease
cannot properly metabolize cholesterol and other lipids. Consequently, excessive
amounts of cholesterol accumulate in the liver and spleen and excessive amounts
of other lipids accumulate in the brain. Symptoms may include dementia,
confusion, and problems with learning and memory. These diseases usually begin
in young school-age children but may also appear during the teen years or early
adulthood.
Batten disease is a fatal, hereditary disorder of the nervous system that
begins in childhood. Symptoms are linked to a buildup of substances called lipopigments in the body's tissues. The early symptoms include personality and
behavior changes, slow learning, clumsiness, or stumbling. Over time, affected
children suffer mental impairment, seizures, and progressive loss of sight and
motor skills. Eventually, children with Batten disease develop dementia and
become blind and bedridden. The disease is often fatal by the late teens or
twenties.
Lafora body disease is a rare genetic disease that causes seizures, rapidly
progressive dementia, and movement problems. These problems usually begin in
late childhood or the early teens. Children with Lafora body disease have
microscopic structures called Lafora bodies in the brain, skin, liver, and
muscles. Most affected children die within 2 to 10 years after the onset of
symptoms.
A number of other childhood-onset disorders can include symptoms of dementia.
Among these are mitochondrial myopathies,
Rasmussen's encephalitis,
mucopolysaccharidosis III (Sanfilippo syndrome), neurodegeneration with brain
iron accumulation, and leukodystrophies such as
Alexander disease, Schilder's
disease, and metachromatic leukodystrophy.
Next: What Other Conditions Can Cause Dementia? »
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