Second edition

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266 Other major syndromes
herpes simplex encephalitis may present in this fashion
(Haymaker 1949; Kennedy 1988; Williams and Lerner
1978), and seizures have also been noted during infectious
mononucleosis (Gautier-Smith 1965; Silverstein et al. 1972).
In those who survive, seizures may occur as sequelae
(Marks et al. 1992), and this appears to be especially likely in
cases in which the acute phase was characterized by seizures
(Annegers et al. 1988) and when there are other sequelae
indicative of cerebral damage (Pomeroy et al. 1990).
In this regard, it may also be noted that acute disseminated
encephalomyelitis, occurring in the days or weeks
following a viral illness, may also be characterized by
seizures (Paskavitz et al. 1995; Tenenbaum et al. 2002).
IN ASSOCIATION WITH CERTAIN DEMENTING OR
NEURODEGENERATIVE DISORDERS
Although partial and grand mal seizures may occur secondary
to various dementing or neurodegenerative disorders,
overall these disorders account for only a small
minority of all seizure cases; furthermore, relative to other
symptoms caused by these disorders, the seizures play but
a minor role in the overall clinical picture.
Multi-infarct dementia may be associated with seizures
at any point in its evolution (Rosenberg et al. 1979); in
Alzheimer’s disease, however, seizures typically occur only
late in its course, and long after the dementia is well-established
(Amatniek et al. 2006; Goodman 1953; Hauser et al.
1986; Romanelli et al. 1990).
Neurosyphilis, in particular general paresis of the insane
(GPI), may cause either grand mal or partial seizures, most
especially simple partial seizures with a Jacksonian march
(Merritt et al. 1932; Storm-Mathisen 1969). Importantly,
the Jarisch–Herxheimer reaction to penicillin treatment
may also be characterized by seizures (Hahn et al. 1959;
Zifko et al. 1994).
AIDS dementia may, late in its course, be accompanied
by seizures (Navia et al. 1986a). Other disorders, often seen
in association with AIDS, must also be considered, including
progressive multifocal leukoencephalopathy and toxoplasmosis
(Navia et al. 1986b; Porter and Sande 1992).
Various movement disorders, typically accompanied by
dementia, may also cause partial or grand mal seizures,
notably the choreiform disorders dentatorubropallidoluysian
atrophy (Porter et al. 1995; Warner et al. 1995),
choreoacanthocytosis (Feinberg et al. 1991; Hardie et al.
1991; Lossos et al. 2005) (which may present with complex
partial seizures years before the onset of chorea [Al-Asmi
et al. 2005]), and the McLeod syndrome (Danek et al. 2001).
In the case of seizures occurring in a young person with a
movement disorder (with chorea, parkinsonism, tremor),
consideration must also always be given to Wilson’s disease
(Dening et al. 1988). Spinocerebellar ataxia, characterized by
a slowly progressive ataxia, in certain of its types, may also
cause seizures (Grewal et al. 2002; Rasmussen et al. 2001)
Cerebrotendinous xanthomatosis, typified by a combination
of either mental retardation or dementia with
Achilles tendon enlargement or cataracts, may also cause
seizures (Fiorelli et al. 1990).
Cerebral amyloid angiopathy, classically causing lobar
hematomas and, in some cases, a dementia, may also manifest
with simple partial seizures, which may occur before
any lobar hemorrhages and either before or concurrent
with a dementia (Greenberg et al. 1993).
Creutzfeldt–Jakob disease, typically characterized by
dementia or delirium with myoclonus, may also, albeit
uncommonly and late in the course of the disease, cause
partial or grand mal seizures (Brown et al. 1986).
Granulomatous angiitis, or isolated angiitis of the central
nervous system, presents subacutely with headache,
which is quite prominent, and delirium, and may, in a
minority, be accompanied by seizures (Vollmer et al. 1993).
Certain degenerative disorders of relatively early onset,
from childhood to early adult years, may also cause partial
or grand mal seizures, including metachromatic leukodystrophy
(Alves et al. 1986; Betts et al. 1968a; Hageman et al.
1995; Haltia et al. 1980; Lima et al. 2006; Rauschka et al.
2006), adrenoleukodystrophy (Moser et al. 1984), pantothenate
kinase-associated neurodegeneration (Rozdilsky
et al. 1968), juvenile Huntington’s disease (Campbell et al.
1961; Siesling et al. 1997), and, especially, subacute sclerosing
panencephalitis (Koehler and Jakumeit 1976; Kornberg
et al. 1991; Prashanth et al. 2006).
CONGENITAL DISORDERS
Mental retardation, of severe or profound degree, is commonly
associated with seizures (Steffenburg et al. 1996),
and over one-third of patients with autism will also have
seizures, which may be seen both in those with and without
retardation (Danielsson et al. 2005; Gillberg 1991; Olsson
et al. 1988).
Various specific congenital disorders, most associated
with mental retardation, also cause seizures, with each one
being marked by various distinctive features.
Down’s syndrome, typified by a variable degree of mental
retardation and a characteristic dysmorphism with narrowed
palpebral fissures, epicanthal folds, and a small mouth, often
with a large protruding tongue, may be accompanied by partial
or grand mal seizures in adults (Pueschel et al. 1991), the
proportion affected rising from about one-tenth of young
adults, to about one-half of those over 50 years old
(McVicker et al. 1994), and to over three-quarters of those
who go on to develop a dementia (Lai and Williams 1989).
Fragile X syndrome, seen generally, but not always, in
males, is typified by a variable degree of mental retardation,
macro-orchidism, and a characteristic dysmorphism, with a
long, narrow face, prominent forehead, and large ears.
A minority of these patients will also have either partial or
grand mal seizures (Finelli et al. 1985; Wisniewski et al. 1985).
Klinefelter’s syndrome, characterized by tall stature and
eunuchoidism in post-pubertal males, may, albeit rarely,
be accompanied by complex partial or grand mal seizures
(Tatum et al. 1998).