Disautonomia Be Misdiagnosed On EEG5

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Epilepsy
&
Behavior
Epilepsy & Behavior 3 (2002) 285–288
www.academicpress.com

Case Report

Temporal lobe epilepsy and postural orthostatic


tachycardia syndrome (POTS)
M. Seeck,a,* O. Blanke,a and S. Zaimb
a
Presurgical Epilepsy Evaluation Laboratory, Program of ‘‘Functional Neurology and Neurosurgery’’ of the Universities Lausanne, Geneva, Switzerland
b
Division of Cardiology, University Hospital of Geneva, Geneva, Switzerland
Received 19 November 2001; received in revised form 25 February 2002; accepted 7 March 2002

Abstract

We describe a 20-year-old woman suffering from right temporal epilepsy, behavioral disorder, and a complaint of paroxysmal
palpitations accompanied by anxiety. Detailed cardiac evaluation revealed that the palpitations were due to episodes of marked
sinus tachycardia secondary to a concomitant postural orthostatic tachycardia syndrome (POTS) and not of psychogenic origin as
initially thought. Treatment with a beta-blocker resulted in the disappearance of palpitations and the associated anxiety. This is the
first report of the coexistence of partial epilepsy and POTS. The recognition of such a syndrome in epileptic patients is important in
order to offer appropriate therapy. Ó 2002 Elsevier Science (USA). All rights reserved.

Keywords: Epilepsy; Seizures; Heart; Postural orthostatic tachycardia syndrome (POTS); Temporal lobe epilepsy, psychogenic

1. Introduction 2. Case report

Sinus tachycardia is the usual cardiac arrhythmia The patient is a 20-year-old right-handed woman, of
associated with complex partial and generalized epilep- African origin, with epileptic seizures since the age of 9
tic seizures [1], although other arrhythmias such as se- who underwent an initial evaluation shortly after ar-
vere sinus bradycardia, prolonged sinus arrest, and riving in Switzerland at the age of 10. There was no
complete heart block during either the ictal or postictal family history of epilepsy or of psychiatric disorders. It
phase have also been noted [2–4]. In this report we de- was also learned that she had convulsions during a fe-
scribe a young female patient who was initially diag- brile illness at the age of 3, secondary to cerebral ma-
nosed to have a co-occurrence of epileptic and laria. Her seizure semiology (experiential phenomena
psychogenic seizures and, in addition, paroxysmal sup- followed by complex partial seizures) suggested a focal
raventricular tachycardias that sometimes accompanied onset, although several electroencephalograms were
the seizures. Detailed neurologic and cardiac evaluation normal. Carbamazepine was subsequently prescribed
revealed that the patient had temporal lobe epilepsy and but the patient was non-complaint and lost to follow-up.
coexisting postural orthostatic tachycardia syndrome She was reevaluated at the age of 18 after presenting to
(POTS) with episodic, very rapid sinus tachycardias, one the emergency room with several hours of prolonged
of the manifestations of POTS, and not an independent complex visual and auditory hallucinations as well as
arrhythmia. To our knowledge, this is the first case in cardiac palpitations. Her interim history was notewor-
which both conditions are simultaneously present in the thy for several suicide attempts as well as of child abuse
same patient. by a close family member. The patient was thought to be
experiencing either nonconvulsive partial status epilep-
* ticus or psychotic decompensation in the context of a
Corresponding author. Present address: Department of Neurology,
H^
opital Cantonal Universitaire de Genève, 24 rue Micheli-du-Crest,
posttraumatic stress syndrome. MRI showed right hip-
CH-1211 Genève 14, Switzerland. Fax: +41-22-372-83-40. pocampal sclerosis, confirmed by volumetric measure-
E-mail address: [email protected] (M. Seeck). ments. EEG revealed bitemporal spike and sharp slow

1525-5050/02/$ - see front matter Ó 2002 Elsevier Science (USA). All rights reserved.
PII: S 1 5 2 5 - 5 0 5 0 ( 0 2 ) 0 0 0 0 3 - 3
286 M. Seeck et al. / Epilepsy & Behavior 3 (2002) 285–288

wave discharges. A diagnosis of temporal lobe epilepsy, control the seizure disorder. No EEG changes in the
with possible previous febrile convulsions, and post- intracranial electrodes were observed during several
traumatic stress syndrome was established. Carb- supraventricular tachycardia episodes. Consistent with
amazepine, at a lower titration rate, was again the phase 1 results, the left temporal focus predominated
prescribed and appeared to be effective but was dis- on interictal EEG. No actual seizures were recorded,
continued because of neutropenia. Several other drugs, however, despite sleep and prolonged drug withdrawal.
alone and in combination, were subsequently tried on an After hospital discharge, the patient, complaining of
outpatient basis but either were ineffective or caused an ill-defined malaise that she associated with the beta-
unacceptable side effects. blocker, stopped taking the medication and returned
The patient was therefore hospitalized again for de- with a complaint of recurrence of multiple self-termi-
termination of the suitability of surgical treatment of her nating and intolerable episodes of palpitations and
seizure disorder and underwent additional studies, in- anxiety. She was therefore admitted for a diagnostic
cluding PET, neuropsychological testing, and long-term cardiac electrophysiology study (EPS), for the purpose
video-EEG monitoring. Two types of episodes were of confirming the presence of an atrial tachycardia, with
observed. The first were episodes labeled nonepileptic radiofrequency ablation of the arrhythmia to follow. No
psychogenic in origin during which she was unrespon- supraventricular arrhythmias could be induced during
sive, displayed profound breathing and arching, with EPS but periodic sudden spontaneous accelerations of
eyes closed, and was resistant to passive eye opening. the sinus rate from 100 to 140 min, within 5 s, were ob-
These events lasted up to 1 h, with no changes in the served while the patient was supine on the examination
EEG. Of concern during these episodes was the occur- table, in her baseline state. A head-up tilt-table study
rence of an assumed atrial tachycardia at rates up to was scheduled after it was also noted that the patient
200/min lasting up to 10 min as noted on monitored experienced a similarly rapid increase in sinus rate, as
single-lead ECG. The second type of episodes consisted well as her usual anxiety and palpitation symptoms
of complex partial seizures with right anterior temporal when getting up from the supine position. The head-up
onset in the EEG. Ictal EEG was concordant with the tilt-table study revealed a striking acceleration of sinus
side of the hippocampal sclerosis and with the presence rhythm immediately after tilting from the horizontal to
of a right temporal hypometabolism on PET, but inte- the 60° vertical position (Fig. 1). The sinus rate in-
rictal EEG showed mainly left temporal spikes or sharp creased from 98 min supine to 190 min within 30 s. The
waves (90%). Moreover, postictal neuropsychological blood pressure actually increased during this time from
testing revealed both verbal and visuospatial memory 123/83 supine to 156/98 at the end of 60 s. There was
loss in comparison to her interictal performance. The also complete reproduction of the patient’s symptom
diagnosis of right temporal epilepsy with significant left complex of palpitations and lightheadedness. The sinus
temporal dysfunction was thus retained. A comprehen- rate also decreased abruptly at the end of the study
sive psychiatric evaluation was also performed and an (3 min), from 170 to 112 min within 10 s of returning to
additional diagnosis of Dissociative Disorder (300.15 the horizontal position. The early rapid and marked
DMS-IV, American Psychiatric Association, 1994) was increase in heart rate with a blood pressure that re-
made. On the basis of these findings, the decision was mained at the baseline level or higher during the study
made to pursue drug treatment rather than surgery. along with the accompanying symptoms made it evident
During the follow-up visits the patient began to
complain increasingly of palpitations associated with
significant anxiety and sometimes lightheadedness. She
had first noticed it 5 years earlier but the frequency had
recently increased. A 24 h-ambulatory ECG recording
showed sinus rhythm with numerous periods of what
seemed to be an atrial tachycardia at rates up to 200/
min, with clear atrial activity associated with and driv-
ing each QRS complex in a 1:1 relationship. A stress test
was subsequently performed, during which she dis-
played upward eye deviation with preserved conscious-
ness, and was normal with a peak (sinus rhythm) heart
rate of 179 min. Transthoracic echocardiography was
normal. A beta-blocker, metoprolol, 50 mg/day was Fig. 1. Graph showing heart rate (HR) and blood pressure (BP)
changes during head-up tilt-table testing. The table is tilted up to 60°
prescribed for presumed atrial tachycardia. Invasive
immediately after Time 0 and brought back to horizontal position
investigations with foramen ovale and scalp recordings immediately after the 3-min reading. H, horizontal position, BP s,
were also performed in parallel with the cardiac inves- systolic blood pressure (diamonds), BP d, diastolic blood pressure
tigations because of the failure of drug therapy to fully (triangles); HR, heart rate (circles).
M. Seeck et al. / Epilepsy & Behavior 3 (2002) 285–288 287

upright tilt, heart rate of 120 min within 5 min of


standing or upright tilt, development of orthostatic
symptoms without orthostatic hypotension, and absence
of a known cause of autonomic neuropathy [6,7]. The
present patient met these criteria; i.e., her rate increase
was dramatic and even greater than the reported in-
crease (from 79  13 to 112  28; mean  SD bpm) ob-
served by Sandroni et al. [5] The same group also noted
the likely benefit of beta-blockers which were introduced
successfully in our patient.
As mentioned earlier, our patient was diagnosed to
Fig. 2. Graph showing the variation in heart rate (HR) and blood
have a dissociative disorder and her complaints of pal-
pressure (BP) during head-up tilt-table testing after 10 weeks of biso-
prolol treatment. The table is tilted up to 60° immediately after Time 0 pitations were thought to be of psychiatric origin. In
and brought back to horizontal position immediately after the 4-min reality, the episodes labeled as psychogenic seizures ac-
reading. For abbreviations, see Fig. 1. tually proved to be her behavioral reaction to the sinus
tachycardia episodes as part of POTS. It is in fact not
that this patient had POTS. The patient was placed on uncommon for POTS patients to be thought to have an
another beta-blocker, bisoprolol, 5 mg/day, which she anxiety or panic disorder [8]. Moreover, some of the
could tolerate, and she has not experienced any recur- symptoms, such as lightheadness and palpitations, are
rences of palpitations since. In keeping with the clinical also experienced during epileptic auras. Given the sim-
improvement, a repeat head-up tilt-table study 10 weeks ilarity of symptoms in psychiatric, cardiologic, and ep-
later, on therapy, revealed a sinus rate at 75 min supine ileptic disorders, the existence of an additional etiology
with a maximum of 113 min during the fourth minute in the cardiovascular domain needs to be considered in
(Fig. 2). There was no significant change in the blood patients presenting with an unusual symptomatology to
pressure, which was 132/76 at baseline and 139/77 at the offer optimal treatment.
fourth minute. The patient remained asymptomatic While the diagnosis and therapeutic management of
throughout the study as well. In retrospect, based on the POTS have become increasingly well defined, the neu-
dynamics of the sinus node behavior noted on subse- rophysiologic origin of the disease is not so clear. Sug-
quent ECG recordings and comparison of ‘‘p’’-wave gested mechanisms of POTS include partial sympathetic
morphologies among the different ECG tracings, it also denervation in the legs, excessive venous pooling, beta-
became clear that the initial ‘‘atrial tachycardias’’ noted receptor hypersensitivity, alpha-receptor hyper- or
in this patient were actually episodes of sinus tachycar- hyposensitivity, and altered sympathetic–parasympa-
dia (as part of POTS). thetic balance [6]. Brainstem dysfunction has also been
suspected [6], but so far, consistent with our findings, no
evidence of a cortical dysfunction has emerged. No EEG
3. Discussion changes in scalp and foramen electrode recordings were
noted during the tachycardias in this patient despite
We report the first case of coexistence of temporal signs of bitemporal damage, thus making a cerebral
lobe epilepsy and postural orthostatic tachycardia syn- origin unlikely.
drome. POTS is a type of orthostatic intolerance char-
acterized most frequently by the occurrence of
orthostatic-type symptoms such as palpitations, light
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