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272

Section 3

Organic syndromes of schizophrenia: other neurological disorders

Anthony Feinstein

Facts box

consequence of which is a relative lack of
r

Wallerian degeneration of fiber tracts

The major demyelinating disorder is multiple
2. Infiltration of inflammatory cells in a perivascular
sclerosis (MS) with three other conditions:
distribution

neuromyelitis optica (Devic’s Disease), acute
3. Distribution of lesions that are primarily in the
disseminated encephalomyelitis, and acute
white matter
[1]

and subacute necrotizing hemorrhagic

encephalomyelitis making up the quartet.

r

If these three principles are obeyed, the clas-Although the data have been inconsistent,
sification is dominated by multiple sclerosis (MS)
recent evidence suggests that psychosis is
with three other conditions: neuromyelitis optica
more likely to occur in MS than by chance
(Devic’s Disease), acute disseminated encephalomyeli-association.

r

tis, and acute and subacute necrotizing hemorrhagic
A shared etiology is unlikely to explain the
encephalomyelitis making up the quartet. Absent from
association of demyelinating disorders and
the list are disorders in which myelin loss is prominent,
schizophrenia.

for example, anoxic encephalopathy, vascular occlu-r
Brain lesions superimposed on a premorbid
sion as in Binswanger’s Disease, progressive multifo-vulnerability (genetic, developmental,
cal leukoencephalopathy, central pontine myelinolysis,
premorbid psychiatric history) offer a more
Marchiafava Bignami Disease, and human immune
plausible explanation for the association.

virus HIV-associated encephalopathy. These disorders
r
There are no empirically based treatment
have been removed from the classification because
studies of psychosis associated with MS.

their etiology has been established. Perhaps more con-r
The increased prevalence of psychosis in
troversial is the omission of the chronic progressive
demyelination challenges the notion of

leukodystrophies of childhood and adolescence, on the
psychosis as a primarily cortical

basis that their cardinal pathological feature is not so
phenomenon.

much a loss (demyelination) but rather an abnormality (dysmyelination) of myelin
[1].
Putting aside the
debate over the choice of a correct prefix, it is ger-mane to note that one of these disorders, metachromatic leukodystrophy, is associated with a rate of
Demyelinating disorders

psychosis that may exceed all other neuropsychiatric
Before discussing psychotic disorders that may arise
disorders
[2]
– this association is discussed elsewhere
from demyelination, it is important to comment
in this book.

briefly on the classification of demyelinating disor-This brief introduction to taxonomy helps frame
ders, a topic not without controversy. To satisfy the
the content of this chapter, which consists primar-descriptor “demyelinating,” three criteria should be
ily of a review of the literature pertaining to psy-met:
chosis associated with MS and a shorter discussion
on psychosis secondary to metachromatic leukodys-1. Destruction of the myelin sheaths of nerve
trophy. The chapter concludes with some thoughts on
fibers with relative sparing of other elements of
the pathogenesis of psychosis in relation to pathology
273

the nervous tissue, that is, axons and nerve cells, a
affecting the cerebral white matter.

Organic Syndromes of Schizophrenia – Section 3

Behavioral abnormalities in

drome, and affective psychosis. Applying these criteria
multiple sclerosis

to their literature review, they came across 39 reports,
a frequency judged not to exceed chance expectation.

Multiple sclerosis is the most common cause of neu-The view that psychosis seldom occurs with MS

rological disability in young and middle-aged adults.

has been cautiously supported by studies investigating
Behavioral sequelae are frequent. The lifetime preva-the number of MS patients found in large, inpatient
lence of major depression approaches 50%, bipolar
psychiatric populations. Percentages from state hos-affective disorder occurs twice as often than in the
pitals in Massachusetts (.07%), Manhattan (.05%),
general population, and pathological laughter and cry-and Queensland (.06%) are similar and do not exceed
ing affects up to 10% of patients. In addition, approxi-chance probability, but may be misleadingly low
mately 40% of community-living MS patients are cog-because of a greater community tolerance for mental
nitively impaired
[3].

disturbance in the presence of MS or alternative
The association between MS and psychosis has
admissions to hospitals caring for the physically
until recently been considered uncommon, which
disabled
[4].

helps explain the paucity of research devoted to the
A more recent study has, however, challenged pre-topic. Given that the lifetime prevalence for a psychotic
vailing assumptions. Population-based evidence from
illness such as schizophrenia is approximately 1% and
Alberta, Canada suggests psychosis in MS patients is
for MS 0.1 to .01% (varying according to latitude), the
more common than was previously thought
[5].
As
two disorders can be expected to appear together by
part of universal health care insurance in Alberta,
chance every 0.5 to 1 in 100,000 cases. Therefore, to
all subjects seen by physicians are given diagnoses
support the specific notion of an MS psychosis that is
coded by the International Classification of Diseases,
distinct from schizophrenia, factors regarding descrip-Ninth Edition Clinical Modification (ICD-9-CM). In
tive, predictive, and constructive validity should be
2002, 10,367 of the 2.45 million residents of this Cana-present.

dian province over 15 years of age were found to
have multiple sclerosis, giving an estimated preva-

Psychosis secondary to

lence of 330 per 100,000, in keeping with Alberta’s
multiple sclerosis

historically high prevalence rate. The authors also
looked for the co-occurrence of two ICD-9-CM psychiatric diagnoses in these MS patients. The first was
Prevalence

“nonorganic/nonaffective psychoses” (which included
Most reports of MS and psychosis are single case stud-schizophrenia-spectrum disorders, delusional disories, with the earliest dating from the nineteenth cen-ders, and other nonorganic psychoses) and the sec-tury. In their comprehensive review of “schizophrenia-ond category was broadly defined as organic psychotic
like psychoses associated with organic disorders of the
disorders (which comprised drug-induced psychotic
central nervous system (CNS),” Davison and Bagley
disorders, other transient organic psychotic disorders,
[4]
devoted a section to demyelinating disease. They
and other organic psychotic disorders). Care was taken
reviewed every published report (irrespective of lan-to exclude patients with dementia and alcohol-related
guage) of multiple sclerosis that occurred concurrently
psychoses.

with a psychotic illness that fulfilled the 1957 World
Results were given separately for each of these two
Health Organization (WHO) criteria for schizophre-large categories and were stratified according to the
nia. Given their belief that the presence of coarse
following age groups: 15 to 24; 25 to 44; 45 to 64;
cerebral pathology would render some signs and
and 65
+
. Consistent findings emerged across all age
symptoms invalid, they excluded catatonia, autism,
groups and in both psychiatric categories, namely, that
and change in personality from the WHO guidelines
the prevalence of psychosis in MS patients significantly
and were left with the following: the presence of an
exceeded that reported in patients without MS.