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347

Section 3

Organic syndromes of schizophrenia: genetic disorders related to SLP

28Huntington’sDiseaseandrelated

disorders and their association

with schizophrenia-like psychosis

Perminder S. Sachdev

Facts box

[4].
Pockets of very high prevalence of HD have been
r

described in places like Tasmania in Australia and
The development of suspiciousness and ideas
the Moray Firth area of Scotland
[5].
It was one such
of reference is seen in many patients with
pocket in the Venezuelan villages of Barranquitas and
Huntington’s Disease (HD).

Lagunetas that the early work toward the discovery of
r
Rates of schizophrenia-like psychosis (SLP)
the HD gene was carried out by the U.S.-Venezuela
in HD ranging from 5% to 16% have been

Huntington’s Disease Collaborative Research Project
reported.

[6].
The linkage of the HD gene to chromosome 4p in
r
It is not uncommon for some patients with
1983
[7]
and its eventual discovery as a trinucleotide
HD to have received a diagnosis of

repeat 10 years later
[8]
are landmark events in the
schizophrenia, possibly for years, before their
history of neurogenetics.

accurate diagnosis.

r
The HD patients who develop psychosis do
Clinical features

not have repeat numbers different from those
without psychosis.

Age of onset

r
It is possible that, despite their differences,
The usual age of onset is in the fourth or fifth decade,
HD patients may develop neuropsychological
with the median age in the mid-40s. However, onset
deficits that resemble those in schizophrenia
has been described in early childhood as well as in the
and this might provide the substrate for the
80s. The disease tends to be more severe if the onset
development of psychotic symptoms in HD.

is early, and premonitory psychiatric features are more
r
Psychosis is uncommon in spinocerebellar
common in such cases. The age of onset is also some-ataxia, but other psychiatric disorders are
what related to the motor features, with striate rigid-common.

ity prominent in the early 20s, chorea in midlife, and
r
Triplet repeats do not appear to have a major
intention tremor more common for a later onset. In
role in the genetics of schizophrenia, but a
siblings, onset seems to be closer together, but still
minor role in a minority of cases cannot be
with considerable variability. Onset before the age of
ruled out.

20 occurs in about 10% of cases and is regarded as
“juvenile onset.” Age of onset is said to show “anticipation,” in other words, the disorder becomes manifest at
Huntington’s Disease (HD) is a progressive neu-an earlier age as it is passed on to the next generation,
rodegenerative disease with a classical autosomal
especially for paternal transmission.

dominant pattern of inheritance. It has conferred
eponymous fame on George Huntington who

described the typical clinical triad of movement dis-

Signs and symptoms

order, cognitive impairment, and psychiatric features,
The initial symptoms are equally likely to be neurologi-and emphasized its familial nature
[1].
The disorder
cal or psychiatric, and no general rule can be described
is uncommon, with a reported prevalence of about 4

for their chronology. Typically, choreiform movements
to 7 per 100,000 in most Western countries
[2, 3, 4]

are the first manifestation and cognitive and psy-but much lower rates in Asian and African countries
chiatric symptoms follow. However, the psychiatric
348

Chapter 28 – Huntington’s Disease and related disorders

symptoms may sometimes precede the movement disin the absence of typical cortical disturbances such as
order or cognitive dysfunction by many years.

dysphasia, agnosia, and apraxia has suggested the term
“subcortical dementia” to describe it. In late stages,
Neurological symptoms

the patient is severely impaired and may show akinetic mutism. The degree of cognitive impairment is
The choreiform movements initially appear in the
related to the duration of the disease and degree of
hands and face and make the patient appear clumsy
caudate nucleus and generalized atrophy. Mild cogni-and nervous. These are usually in the form of twitch-tive deficits have been reported in asymptomatic car-ing and grimacing. Gradually, these spread to the
riers
[15],
and an inverse relationship between cogni-rest of the musculature, making movements jerky and
tive function and the length of triplet repeats has been
rapid, and, in the late stages, the patient is contin-noted
[16].