Medical Internal Medicine
Resume:
Blackwell Munksgaard ****: **: 55 57
Journal compilation # 2006 Blackwell Munksgaard
Printed in Singapore. All rights reserved
ACTA NEUROPSYCHIATRICA
CASE REPORT
A case of subacute sclerosing panencephalitis
presenting as depression
Datta SS, Jacob R, Kumar S, Jeyabalan S. A case of subacute sclerosing Soumitra Shankar Datta1, Rajesh
Jacob1, Sudhir Kumar2, Susan
panencephalitis presenting as depression.
Acta Neuropsychiatrica 2006: 18:55 57. # Blackwell Munksgaard, 2006 Jeyabalan1
1
Department of Psychiatry, Christian Medical College, Vellore
Summary: Subacute sclerosing panencephalitis (SSPE) is rare in adult 632002, Tamil Nadu, India; and
2
patients. Clinical presentation in intial phases of SSPE may be non- Department of Neurology, Christian Medical College, Vellore
632002, Tamil Nadu, India
specific leading to diagnostic delay. We present a 24-year-old patient
with depressive syndrome of five months duration prior to the onset of
Keywords: adult-onset; depression; electroencephalography; psy-
typical features of SSPE, which is a rare presentation. This patient had
chiatric symptoms; Sertraline; subacute sclerosing panencepha-
responded partially to Sertraline, for a brief period, before he was litis (SSPE)
diagnosed to have SSPE. This case illustrates affective symptoms can be
the presenting features of SSPE in adults. Correspondence: Dr Rajesh Jacob MBBS, MD, Department of
Psychiatry, Christian Medical College, Vellore 632002, Tamil Nadu,
India, Tel: 91-416-*******; Fax: 91-416-*******;
E-mail: abp9la@r.postjobfree.com
Subacute sclerosing panencephalitis (SSPE) is Here, we present a case of an adult onset SSPE
very rare in developed countries but is still com- that initially presented with a depressive syndrome
mon in developing countries. Saha et al. (1) for 5 months till the onset of typical myoclonic
reported an annual incidence of 21 per million jerks, which is a rare presentation.
population in India. SSPE usually presents in A 24-year-old man was referred to the depart-
childhood and adolescence. The initial symptoms ment of psychiatry from the internal medicine
are subtle and include mild intellectual deteriora- department with the complaints of a 5-month his-
tion and behavioural changes without any appar- tory of feeling sad, loss of interest in pleasurable
ent neurological signs or findings. Parents and activities, increased fatigue, lethargy, somatic
teachers may notice progressive deterioration in complaints and insomnia. In addition to the
scholastic performance. above depressive syndrome, he had two episodes
It is uncommon after 18 years of age, and the of transient loss of awareness of his surroundings.
disease has a more aggressive course in adults. Both these episodes occurred in the presence of
Patients with adult onset SSPE present at a mean others and inside his house. There were no episodes
age of 25.4 years (range 20 35 years). Clinical during sleep nor were there any injuries sustained
features at presentation usually include cognitive during these episodes. There was no history sug-
decline, behavioural changes and myoclonic gestive of myoclonic jerks, urinary incontinence,
jerks. Features later in the disease include decere- lip-smacking movements or any other seizure
brate and decorticate posturing, dysphagia, dys- equivalents. Detailed neurological examination
arthria and coma (2). did not reveal any focal neurological signs. Other
The diagnosis is often made at a later stage and systemic examinations were also within normal
may be missed in the initial stages as the signs and limits. A mental state examination revealed a
symptoms are subtle or atypical (3). The diagnosis reduced attention span. The patient s speech was
of SSPE is suspected clinically and confirmed with slow and laconic. Thought content revealed
the characteristic electroencephalogram (EEG) depressive cognitions. Affect was depressed and
findings and cerebrospinal fluid (CSF) measles nonreactive. After an outpatient evaluation in the
antibody titres (4). department of psychiatry, he was diagnosed to be
Blackwell Munksgaard, Acta Neuropsychiatrica, 18, 55 57 55
#
Datta et al.
Fig. 1. Electroencephalography showing typical periodic complexes of subacute sclerosing panencephalitis.
cogwheel rigidity of the upper limbs bilater-
suffering from moderate depression with somatic
ally. Gait showed diminished arm-swing. The
symptoms according to ICD-10 classification.
posture was remarkable for intermittent jerky
Organic causes for his mood symptoms were
movements of the upper limbs, more so of the
suspected. An EEG (Fig. 1) was done to rule out
right side.
temporal lobe epilepsy. The initial EEG was
A repeat EEG revealed bursts of high-amplitude
normal, and the transient falls were attributed
(upto 250 microvolts) sharp and slow wave
to dissociative phenomenon as a part of the
discharges bilaterally and synchronously, occur-
depressive syndrome.
ring at regular intervals of 15 s.
The patient was started on Tab Sertraline
This activity was associated with jerking of
50 mg per day with which his depressive symp-
the right upper and lower limbs. These periodic
toms improved to an extent. He was followed-
complexes were suggestive of fully evolved SSPE.
up as an outpatient on a regular basis once in 2
Computerized tomography of brain was done,
weeks. After 2 months, he presented with repe-
which was normal. Magnetic resonance imaging
titive intermittent jerky movements of the
was not done because of financial constraints for
upper limbs. The jerks were asymmetrical and
the patient.
affected the right side of his body more than
Measles antibody titres in the CSF examined
the left. Because of the persistence and perio-
were strongly positive. A ratio of 1/64 and more
dic nature of the jerks and history of periods
between paired serum and CSF antibodies was
of altered sensorium, the patient was referred
considered diagnostic. Immunofluoroscence was
to the neurology department. Besides higher
used to detect measles antibodies in the CSF.
mental function, an examination revealed
Patient was started on supportive treatment but
that although he was oriented to time, place
he died within 2 months.
and person, he had difficulty in obeying
SSPE is common in developing countries.
complex commands. Recent memory and new
Although it usually affects children and adoles-
learning ability were impaired. Cranial nerve
cents, it can present in adults rarely. The diagno-
examination was notable for hypometric sac-
sis may not be suspected as the initial phase of
cades. Motor system examination showed mild
56
Subacute sclerosing panencephalitis
treatments become available. This report is basi-
behavioural changes, and cognitive decline is
cally to highlight another atypical presentation of
often labelled as functional. Our case had a long
adult-onset SSPE presenting with a depressive
period of predominantly depressive symptoms
syndrome which is a rare presentation in the
for 5 months with no other features suggestive
initial stages. Another atypical feature in this
of cognitive decline or personality change.
case was the rather long duration of depressive
The presence of normal EEG in the early stage
symptoms before the appearance of myoclonic
makes clinicians even more complacent about
jerks. Therefore, clinicians need to have a high
suspecting a diagnosis of SSPE. The typical
index of suspicion in patients presenting with
EEG findings of SSPE appear in stage II of the
these symptoms from areas with high prevalence
disease. EEG in a typical case in stage II is char-
of SSPE for early diagnosis.
acterized by high amplitude 300 1500 microvolts
repetitive polyphasic sharp and slow wave com-
plexes of 0.5 2 s duration, recurring every 4 15 s
Acknowledgements
synchronously with myoclonic jerks (4). EEG in
the end stages of the disease again becomes aty- We thank Dr Prathap Tharyan, Professor of Psychiatry, and
Dr Chandran Gnanamuthu, Professor of Neurology, for their
pical, as the background and sleep activities are
support and guidance.
disorganized, and there is a gradual reduction in
the amplitude till it finally becomes isoelectric.
Our patient had a rapid deterioration and died References
2 months after diagnosis. The natural history of
1. SAHA V, JOHN TJ, MUKUNDAN P. High incidence of
the temporal profile of SSPE is highly variable
subacute sclerosing panencephalitis in South India.
and can be of three types subacute onset with Epidemiol Infect 1990;104:151 156.
duration of illness 1 to 3 years (seen in 80% 2. GARG RK. Subacute sclerosing panencephalitis. Postgrad
of cases), rapid course with death in 3 months Med J 2002;78 (916):63 70.
after diagnosis (seen in 10% of cases) and a 3. DYKEN PR. Subacute sclerosing pan encephalitis: current
status. Neurol Clin 1985;3:179 196.
slowly progressive form with evolution over 4 8
4. COBB WA, HILL D. Electroencephalography in subacute
years (5). sclerosing panencephalitis. Brain 1950;73:392 404.
The importance of recognizing the spectrum of 5. PRABHAKAR S, ALEXANDER M. Subacute sclerosing
potential presentations of SSPE and providing an panencephalitis. In: RAAD A, CHARLES M, eds. Tropical
early diagnosis will increase as more effective neurology. Philadelphia: W.B. Saunders, 1996: 77 93.
57
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