The role of nonpharmaceutic conservative interventions in the treatment and secondary prevention of epilepsy

Nonpharmacologic conservative treatments receive too little attention. Depending on the clinical condition of the patients, they may be used alone or in conjunction with other therapies. Their target is the single seizure rather than the epileptic condition as such. They belong mainly to one of three domains. NONSPECIFIC PREVENTION OF SEIZURES: The first step is the identification of factors facilitating the occurrence of seizures. In the second step, strategies to control these factors are developed. Most common are disturbances of the sleep-wake cycle, especially reduction of sleep. Patients should follow a regular sleep schedule with deviations of not >2 h. Sometimes a sleep calendar is helpful. Night shifts are not compatible with seizure prevention in these cases. Sleep disturbances as a facilitating factor of seizures are particularly common in juvenile idiopathic generalized epilepsies, in which their avoidance is in many cases an indispensable part of the therapeutic regimen, along with appropriate drug treatment. They are the most common precipitating factor in adolescents and adults with a first epileptic [mostly generalized tonic-clonic (GTC)] seizure. In these instances, their avoidance is central to the secondary prevention of epilepsy developing from the single seizure, whereas the prescription of antiepileptic drugs (AEDs) is rarely effective. Other nonspecific facilitators of seizures include uncontrolled use of alcohol and extraordinary stress. Patients must learn how to cope with stressful events. SPECIFIC PREVENTION OF SEIZURES: In reflex epilepsies, specific precipitants of seizures are the targets of interventions. Thus, most patients with primary reading epilepsy begin to have, with prolonged reading, perioral reflex myoclonias, which enable them to stop reading and thus to avoid a GTC seizure. In photosensitive patients, seizures are often precipitated by television. These can be avoided by viewing from a distance and using a remote control, small screens in a well-lit room, and preferably with a 100-Hz line shift. Environmental flicker stimulation often comes unexpectedly, and it is advisable that the patients always wear sunglasses in brightly lighted surroundings. Polarized glasses seem to be more protective than plain sunglasses. If the patient has only photically induced seizures, treatment by specific prevention alone may be sufficient, but if spontaneous seizures also occur, drugs must be given in addition. ARREST OF SEIZURES: Focal seizures that develop sufficiently slowly to leave the patient time to react may be interrupted by "countermeasures." These, again, may be nonspecific (acting by relaxation, concentration, or a combination of both) or specific. The latter are individually tailored, based either on spontaneous experiences of the patient or on the anatomy of ictogenesis. Seizure propagation is blocked when a major part of the neurons involved is activated and not recruitable for spread of the epileptic discharge. Seizure arrest rarely is used alone but usually in combination with partially successful pharmacotherapy.

Primary reading epilepsy: therapeutic efficacy of clonazepam in one case

Primary reading epilepsy is a rare form of reflex epilepsy, in which reading is the specific stimulus evoking attacks. The authors report a case of an 18-year-old man who since the age of 17 presented myoclonic jaw jerking provoked exclusively by reading. In one episode, in which reading was not interrupted, jerks were followed by a generalized convulsive seizure. EEG with routine activating procedures was normal, while EEG recorded during reading showed bilaterally synchronous paroxysmal small-voltage spikes, more prominent in frontocentral regions, coinciding with jaw myoclonus. Complete clinical seizures control and EEG normalization were achieved with clonazepam 2 mg daily in a 24-month follow-up.

Reading and language-induced epilepsy

Primary reading epilepsy
Primary reading epilepsy usually begins when the person is between 12 and 25 years old. In this condition, seizures are provoked only by reading, and people do not have seizures at other times. Usually, while reading, the person’s jaw clicks or jerks and, if she keeps reading, she may have a generalized tonic-clonic seizure. Other seizure types may also occur. People with this syndrome often have a family history of epilepsy, and cases of reading epilepsy that run in families have been reported. Primary reading epilepsy may be a specific form of language-induced epilepsy (see below).

Secondary reading epilepsy
Secondary reading epilepsy is very similar to primary reading epilepsy, except that people with this condition may also have unprovoked seizures.

Language-induced epilepsy
In language-induced epilepsy, stimuli such as writing, typing, listening to speech, singing, or reciting may trigger seizures. People with this syndrome have jaw jerks with abnormal EEG activity.

Some symptomatic epilepsies may also result in reading- or language-induced seizures.

Indian Epilepsy Association

Indian Epilepsy Society

http://www.epilepsyindia.org/indianepilepsysociety/index.htm

International League Against Epilepsy (ILAE)

The International League Against Epilepsy (ILAE) is the world’s preeminent association of physicians and other health professionals working towards a world where no persons' life is limited by Epilepsy. Its mission is to provide the highest quality of care and well-being for those afflicted with the condition and other related seizure disorders.

The League aims:

To advance and disseminate knowledge about epilepsy
To promote research, education and training
To improve services and care for patients, especially by prevention, diagnosis and treatment

Inheritance of reading epilepsy

Primary reading epilepsy was diagnosed by special electroencephalo- graphic (EEG) studies in an adolescent youth after his first grand mal seizure. Similar studies of members in three generations of his family showed primary reading epilepsy in his first cousin and in two of his siblings. The disorder in the siblings had not been noticed by parents or teachers and probably would have remained undetected without the use of special techniques. This pedigree buttresses other reported evidence for autosomal dominant transmission of reading epilepsy. The centrencephalic EEG trait also is present in some members of this family. The possible implications of this association are discussed.

Inheritance of reading epilepsy
RICHARD F. DALY, M.D. and FRANCIS M. FORSTER, M.D.
Department of Neurology, University of Wisconsin Center for Health Sciences, Madison, Wisconsin.

NEUROLOGY 1975;25:1051

Reading Epilepsy

For a long time it has been known that seizures could
be evoked in certain epileptic individuals by a discrete
physiologic or psychologic stimulus. The term reflex
epilepsy is reserved for this small subgroup. Forster(1)
has classified these seizures in accordance with their
evocative stimuli into five types: - (1) Visual-flickering
light, visual patterns & specific colors especially red,
leading to rapid blinking or eye closure; (2) Auditorysudden
unexpected noice (startle), specific sounds,
musical themes & voices; (3) Somatosensory-either a
brisk unexpected tap or sudden movement after sitting
or lying still or a prolonged tactile or thermal stimulus to a
certain part of the body (Hot Water Epilepsy); (4) Writing
or reading words or numbers and (5) Eating.
Reading epilepsy is a rare benign, non progressive
syndrome characterized by reading-provoked
sensorimotor symptoms affecting the oral-buccal-lingualfacial
muscles that are involved in reading aloud(2).
However, reading aloud usually not required to trigger
the seizures. As a result, some authorities have
recommended renaming the condition language-induced
Epilepsy(3). Our case was 9 years old male with no
family history, presented as reading epilepsy which was
misdiagnosed for six months as non-organic seizures.
A 9 year old male child used to visit the pediatricians at
different occasions. Always parents complained that most
of the times child starts with abnormal movements of jaw,
face and sometimes myoclonic jerks while reading. He has
3-4 such attacks during school also. Parents and teachers
corelated it as child disintrest toward study. Each attack
used to last for 1-3 minutes. These attacks were more when
he used to read aloud and specifically English language. He
had about twenty attacks in six months time and this has
disturbed the child including his studies. Onexamination child
was well built and neurologically there was no deficit fundus
examination was normal. His BP- 110/70 mm/Hg. & pulse
rate-80/minute, ECG WNL. His biochemical parameters,
sugar, calcium, magnesium, LFT, RFT, haemogram,
blood counts all were normal. His CT scan head was
normal. His EEG was done on 16 channel digital E.E.G.
machine which showed spike wave and polyspike
discharges (Fig. 1).

The child was diagnosed as reading epilepsy and was
put on sodium valproate 300 mg. twice a day. His seizure
completely subsided and now he is doing well in his studies
and he is no more afraid of reading.
Reading epilepsy is a rare, benign non-progressive
syndrome, characterized by reading-provoked
sensorimotor symptoms affecting the oral-buccal-lingualfacial
muscles that are involved in reading aloud (2). This
condition is accompanied by a positive family history of a
similar disorder in as many as one fourth of cases our
patient has no family history and was a sporadic case.
These patients as described b Krishnan et al (4), presents
as myoclonic, jerking or tonic movements of jaw. Some
patients also report with abnormal sensations such as
stiffness, numbness or tightness during the seizures.
Usually disease starts at puberty, the average age of onset
is 17 years with symptoms starting as young as 10 years
of age(4). Cases have been described that overlap
clinically with benign partial epilepsy of childhood
(BPEC), with Juvenile myoclonic epilepsy (JME) and
with absence epilepsy (5, 6,7).
Our case was 9 year old male child who used to get
abnormal movements of jaw, face and myoclonic jerking
while reading English language. All investigations were
normal but detailed history & EEG gave the final diagnosis
and child was treated with sodium valproate and is doing
well and free of seizures.
This rare case has been presented so that clinicians
should be aware and take history properly to reach a
final diagnosis and to treat this benign reflex epilepsy
syndrome effectively.
References
1. Forster FM: Reflex Epilepsy, Behavioral Therapy,
and Conditional Reflexes, Springfield, IL, Charles C
Thomas, 1977.
2. Wolf P., Reading epilepsy. In : Roger J, Bureau M, Dravet
C, Dreifuss F, Perret A, Wolf P, eds. Epileptic Syndromes
in Infancy, Childhood & Adolescence, 2nd edn. London :
John Libbey and Company, 1992 .pp. 281-98.
3. Koutroumanidis M, Koepp MJ, Richardson MP et al. The
variants of Reading Epilepsy. A clinical & video-EEG study
of 17 patients with reading-induced seizures. Brain 1998;
121 : 1409-27.
4. Radhakrishnan K, Silbert PL, Klass DW. Reading epilepsy.
An appraisal of 20 patients diagnosed at the Mayo clinic,
Rochester, Minnesota, between 1949 & 1989, and
delineation of the epileptic syndrome. Brain 1995; 118 :
75-89.
5. Valenti MP, Tinuper P, Cerullo A, Carcangiu R, Marini C :
Reading epilepsy in a patient with previous idiopathic &
focal epilepsy with Centrotemporal spikes. Epileptic
Discord 1999; 1 : 167-171.
6. Wolf P, Mayer T, Reker M. Reading epilepsy. Report of
five new cases and further considerations on the
pathophysiology. Seizure 1998, 7: 271-9.
7. Singh B, Anderson L, al Gashlan M, al Shahwan SA, Riela
AR. Reading-induced absence seizures. Neurology 1995;
45 : 1623-4.