However, today in psychiatry, a
great void exists with regard to seizures: DSM
4 does not address the links of seizures and psychiatry.
In DSM 3 R we had the non-specific and diagnostically
inadequate and inaccurate conditions of organic
delusional disorder, atypical psychosis, organic
hallucinosis or organic mood disorder. In
DSM IV, we have "symptomatic disorders"
and "mental disorders due to a general medical
condition" with seizures having no special
pride of place. Epilepsy has remained a predominantly
neurologic and not psychiatric condition and the
limited numbers of psychiatric epileptologists
worldwide have not allowed this situation to correct
itself.
When is someone epileptic?
A person is only epileptic
when he has seizures recurrently. An epileptic
seizure involves paroxysmal cerebral neuronal
firing producing clinically relevant changes.
Such seizures may or may not produce disturbed
consciousness. Instead, they may manifest with
other perceptual or cognitive or autonomic or
motor alterations or even what to the patient
may be indescribable types of experiences. In
psychiatric presentations, they may manifest as
subtle episodic behavioral, affective or cognitive
changes which most commonly derive from the temporal
lobe: hence the term Temporal Lobe Epilepsy
- an anatomical origin term replaced today by
the functional and broader non-anatomical description
of "complex partial seizures". The most
classical and common epileptic seizures are of
the "grand mal" or generalized "tonic
- clonic" kind. These usually involve relatively
short ( 10-30 seconds ) tonic movements with marked
extension / flexion of muscles but no shaking
and then a longer (15-60 sec) clonic tonic manifesting
as rhythmic muscle group shaking. These movements
may be associated with a phase of laryngeal stridor
due to tonic muscles manifesting as a high pitched
scream sound. Urinary and occasionally fecal incontinence
may occur due to sphincteric change and the seizures
are almost invariably followed by headache, sleepiness
and / or confusion. When preceded by perceptual,
autonomic, affective or cognitive alterations
such seizures are partial seizures secondarily
generalized. These partial seizures are classified
as complex partial when any impairment of consciousness
occurs, and as simple partial when there are focal
features but no change in consciousness. Often
this call is difficult to make. An epileptic patient
who experiences special symptoms lasting seconds
referable to the cerebral cortex or limbic system
such as a buzz in his ear, burning rubber smells,
distortions in size, sudden sweating, or uncontrolled
weeping, sudden right arm jerks, or a rising epigastric
sensation may be having simple partial seizures.
However, if the patient experiences with these
symptoms, a strange sense of depersonalization
or of unreality, or a sudden profound mood change
over seconds, or a disorientation sense or loss
of time or a frank absence of consciousness with
no other manifestation, he may in fact be having
subtle alterations of consciousness and the symptoms
would reflect complex partial seizures.
Some seizures do not have a specific
locus of origin of firing to produce focal features
and only have the tonic clonic movements or transient
impairments of consciousness without movements
(e.g. petit mal absences which are diagnosable
on EEG) or variants
of these (e.g. bilateral myoclonus, tonic or atonic
episodes) are classified as generalized from the
start and therefore called generalized epilepsy.
(The terminology has changed: Previously primary
generalized epilepsy referred to seizures
generalized from the start in the presence of
normal brain function and electroencephalographic
(EEG) background; secondary generalized epilepsy
was also generalized from the start but had abnormal
brain function and background as in mental retardation
with epilepsy. We should distinguish secondary
from secondarily generalized.)
Neuropsychiatrists, in fact, frequently
recognize that marked behavior disturbance may
correlate with paroxysmal discharges in the brain
(particularly the temporolimbic areas) on the
electroencephalogram although these patients would
not be considered to have a seizure disorder by
many neurologists. Many neuropsychiatrists believe
these patients represent a form of episodic
electrical cerebral firing which Dieter Blumer
and I for non-prejudicial reasons have called
"Paroxysmal Neurobehavioral Disorder".
We characterize the individual events as "atypical
spells".
Thus the spectrum of epilepsy and psychiatry is
broader now than it was, and a whole area - possibly
the majority of patients presenting to psychiatrists
- is couched in controversy and in diagnostic
labels that are ambiguous, uncertain, non-existent
or invented post-hoc based on pragmatic experience.
Sometimes episodes may well be
seizure phenomena, although they cannot be proven
to be so on EEG. This is particularly important
when such symptoms as major episodes of mood lability
lasting seconds happen or confusional episodes
occur or when a symptom like an unexplained rising
epigastric sensation occurs on its own. These
can be classified as atypical spells under paroxysmal
neurobehavioral disorder (PND) because the actual
phenomena may not have been proven to be seizures.
Many of these patients respond to anticonvulsant
treatment so that appropriate neuropharmacologic
response is an important diagnostic indicator.
Both proven seizure phenomena and
PND may be sequelae after an acute brain insult
such as closed head injury, vascular episode,
encephalitis, anoxia or tumor but these conditions
may also be mobilized by chronic cerebral exposure
to recreational drugs of abuse like cocaine, LSD,
cannabis and amphetamines. Some epileptics have
loaded family histories of seizures or other episodic
events like subjective paranormal experiences
but for the most part the etiology of the epilepsy
is not easily determined - cryptogenic.
TABLE 1
International League Against Epilepsy
Revised Classification of Epileptic Seizures (1981)
- Partial (focal, local) seizures:
- Simple - motor, somatosensory, autonomic,
psychic
- Complex
- Impaired consciousness at outset
- Simple partial followed by impaired
consciousness
- Partial seizures evolving to generalized
tonic-clonic (GTC)
- Simple to GTC
- Complex to GTC
- Generalized seizures (convulsive or non-convulsive)
-
- Absence seizures
- Atypical absences
- Myoclonic
- Clonic
- Tonic
- Tonic-clonic
- Atonic
- Combinations
- Unclassified epileptic seizures
Epilepsy plus and Epilepsy standard
Most epileptics are healthy. They
do not have any psychiatric stigmata, and the
only difference between them and a general non-epileptic
population is the occurrence of seizures. I have
dichotomized epileptics:
1. Possibly 90% of cryptogenic
epileptics constitute the epilepsy standard
patient: These epileptic patients have no
more psychopathology than the average patient.
The epilepsy standard patients, without additional
psychopathology, are functional within the community,
and should be differentiated from the epilepsy
plus patients.
2. The second group is the epilepsy
plus patient. This minority of epilepsy patients
have significant behavioral or psychiatric abnormalities
relating to the organic aspects of their seizure
disorder. These patients frequently present psychiatrically,
or may be evaluated commonly in an academic setting.
They may have been misdiagnosed or be more resistant
to conventional anticonvulsants alone needing
psychotropics as well. As many as one-half require
hospitalization for this. These patients may have
a broader coarse neurobehavioral syndrome and
may be mentally retarded (one subpopulation therefore
has "secondary generalized epilepsy"). They also
include a group in which psychoses and seizures
coexist (at a frequency 3 to 5 times the general
population incidence). Finally, probably the most
common population with severe psychopathology
is Blumer's interictal dysphoric disorder patients.
These patients require anticonvulsant plus antidepressant
treatment.
These conditions remain controversial,
and particularly so the debate as to whether these
are linked with temporal lobe foci or relate to
seizure disorders in general. The incidence with
temporal lobe foci is certainly higher, but a
number of confounding variables, such as dosage,
number of anticonvulsants prescribed, severity
of seizures, impairments in seizure control and
number of seizure types, may play significant
roles.
The epilepsy plus patient probably
constitutes a very small minority of the total
number of epileptic patients that will generally
present to major centers, and may be intractable,
difficult to manage, and have behavioral disorders
or associated coarse neurobehavioral syndromes.
These are superimposed on, or coexist with the
epilepsy, and their behavioral disturbances, or
psychoses, may or may not be causally related
to their epilepsy. Moreover, the differentiation
should be clinical as well as academic, because
interpretations of psychiatric populations with
epilepsy as having causal links and not just coexistent
conditions, may distort further research.
One other factor distorts interpretations
of patients with epilepsy and psychopathology.
Psychiatric patients are sometimes perceived as
having epilepsy, when in fact they do not. Their
previous labels of epilepsy may relate to a single
seizure, often associated with alcohol or withdrawal
or other medical conditions. Alternatively, they
may have had an acting-out episode, incorrectly
interpreted as a seizure. They may be on anticonvulsant
medication for mood disorder or paroxysmal episodic
behavior disturbance, and the medication may be
misconstrued by a new treating physician as for
seizure control. They may be wrongly on anticonvulsant
medication for black-outs which have not been
proven to be epileptic. Finally, an abnormal EEG
does not make the patient epileptic. It is probably
best to limit epileptic psychiatric patients to
those having a confirmed history of epilepsy associated
with at least two documented seizures which were
not linked to withdrawal phenomena or pyrexia
or other acute events. Conversely, patients who
are epileptic may have periods of altered or impaired
consciousness following on their seizure, with
the resulting confusion producing behavioral disturbances.
This should not be interpreted as psychiatric;
it is clearly a post-ictal confusional state.
Similarly, patients diagnosed with pseudoseizure
phenomena may be having real seizures which have
been misdiagnosed or in addition to their non-epileptic
events.
Possible temporal lobe symptoms
Seizure disorders with behavioral
disturbance, may initially be interpreted as psychiatric
in origin. Many such problems relate to the temporal
lobe of the brain. The features of temporal lobe
epilepsy and non-epileptic dysfunction of the
temporal lobe are so varied and so protean that
it is necessary to classify them. I have suggested
the term
"Possible temporal lobe symptoms"
(PTLSs) relate to features which can be induced
by stimulating areas of the temporal lobe during
neurosurgery. These symptoms only become specific
symptoms of temporal lobe dysfunction if their
occurrence is validated empirically during a seizure
- either through observation or by the electroencephalogram
(hence the word "possible" in possible temporal
lobe seizures). Great care must be taken in interpretation
of such features : For example, using a phenomenological
analysis, I demonstrated that the symptom of deja
vu commonly regarded as symptomatic of temporal
lobe epilepsy indeed had a very special phenomenologic
quality in patients
with temporal lobe epilepsy.
Like many other such focal symptoms, this involves
its association with
post-ictal features
such as sleepiness, headache and clouded consciousness
and its link in time with these features. This
association provides an excellent clue to the
existence of temporal lobe epilepsy. However,
deja vu is a normal phenomenon occurring in 70
percent of the population and unless such phenomenological
detail is obtained, patients' symptomatology may
be misinterpreted. I similarly studied olfactory
hallucinations but a specific type of temporal
lobe epilepsy olfactory hallucination could not
be demonstrated although there were suggestive
features. A
major message, therefore, may be
the relevance of adequately assessing the symptomatology
of patients presenting with epilepsy. It may be
that this is a direction as relevant as electroencephalographic
monitoring. Most of all it reminds us how
slender our interpretations of other related but
different symptoms such as "he experiences strange
smells" might be and the fact that it is critical
to elicit whether these are episodic in quality
and linked with other symptomatology particularly
epileptic or temporal lobe. A written test instrument
designed to screen for such symptoms which I use
in clinical practice is the
INSET
- The
INVENTORY OF NEPPE OF SYMPTOMS OF EPILEPSY
AND THE TEMPORAL LOBE (INSET). The INSET involves
screening for possible temporal lobe, epileptic
and organic symptoms and spells. Thereafter the
symptoms are categorized into several headers
namely nonspecific symptoms, possible and controversial
temporal lobe symptoms, seizure related and other
focal features. The test is based on the subject
and / or his family responding to questions which
are thereafter elaborated in greater clinical
detail. Responses are at two time levels: current
as well as the most common frequency in the remote
past and require the patient to rank frequency
from never through less than yearly to more than
daily (i.e. 0-6). Questions in the INSET have
been based on the earlier Neppe Temporal Lobe
Questionnaire which itself derived from an intensive
literature review on the topic. The INSET plus
medical history is a major determining factor
for whether to order follow-up specialized electroencephalograms
such as
ambulatory EEG.
TABLE 2: POSSIBLE TEMPORAL LOBE
SYMPTOMS (PTLSs)
Controversial PTLSs (CPTLSs)
- severe hypergraphia
- severe hyperreligiosity
- polymodal hallucinatory experience Paroxysmal
(Recurrent) Episodes of:
- profound mood changes within hours
- frequent subjective paranormal experiences
e.g. telepathy, mediumistic trance, writing
automatisms, visualization of presences or
of lights/colors round people, dream ESP,
out-of body experiences, alleged healing abilities
- intense libidinal change
- Uncontrolled, lowly precipitated, directed,
non-amnesic aggressive episodes;
- recurrent nightmares of stereotyped kind
- episodes of blurred vision or diplopia
Not Necessarily Disintegrative
PTLSs (NPTLSs)
Symptoms Not Necessarily Requiring
Treatment Paroxysmal (Recurrent) Episodes of:
- Complex visual hallucinations linked to
other qualities of perception such as voices,
emotions, or time
Any form of:
-
Auditory perceptual abnormality;
-
Olfactory hallucinations;
-
Gustatory hallucinations;
-
Rotation or disequilibrium
feelings linked to other perceptual qualities;
-
Unexplained "sinking," "rising,"
or "gripping" epigastric sensations;
-
Flashbacks;
-
Illusions of distance, size
(micropsia, macroscopy), (micropsia), loudness,
tempo, strangeness, unreality, fear, sorrow;
-
Hallucinations of indescribable
modality.
-
Temporal lobe epileptic deja
vu (has associated ictal or postictal features
{headache, sleepiness ,confusion} linked to
the experience in clear or altered consciousness
)
-
Any CPTLSs which appear to
improve after administration of an anticonvulsant
agent such as carbamazepine.
Disintegrative PTLSs (DPTLSs)
Symptoms Requiring Treatment: Paroxysmal
(Recurrent) Episodes of:
- Epileptic amnesia;
- Lapses in consciousness;
- Conscious "confusion" ("clear" consciousness
but abnormal orientation, attention and behavior);
- Epileptic automatisms;
- Masticatory-salivatory episodes;
- Speech automatisms;
- "Fear which comes of itself" linked to other
disorders (hallucinatory or unusual autonomic)
;
- Uncontrolled, unprecipitated, undirected,
amnesic aggressive episodes;
- Superior quadrantic homonymous hemianopia;
- Receptive (Wernicke's) aphasia.
- Any CPTLSs or NPTLSs with ictal EEG correlates.
Seizure related features ( SZs
)
Any typical absence, tonic or clonic
or tonic-clonic or bilateral myoclonic seizures
in the absence of metabolic, intoxication or withdrawal
related phenomena.
Clearly there is a greater need
to pay attention to unusual episodic symptoms.
This will ultimately lead to a workable classification
and the recognition that certain seizure like
features need be treated by psychiatrists.