A Case of Myoclonic Symptoms After Streptococcal Infection

Case Report /Olgu Sunumu 85
DOI: 10.4274/npa.y5614
A Case of Myoclonic Symptoms After Streptococcal Infection:
Possible PANDAS Variant
Streptokok Enfeksiyonu Sonras›nda Geliflen Myoklonik Belirtilerle Seyreden Bir
Olgu Sunumu: Olas› PANDAS Variant›
Mehmet Fatih CEYLAN, Salih SELEK*, Esra ZEYT‹NC‹**, Haluk SAVAfi***
Kayseri E¤itim ve Araflt›rma Hastanesi, Çocuk ve Ergen Psikiyatrisi Klini¤i, Kayseri, Türkiye
*Harran Üniversitesi T›p Fakültesi, Psikiyatri Anabilim Dal›, fianl›urfa, Türkiye
**Selçuk Üniversitesi T›p Fakültesi, Psikiyatri Anabilim Dal›, Konya, Türkiye
***Gaziantep Üniversitesi T›p Fakültesi, Psikiyatri Anabilim Dal›, Gaziantep, Türkiye
ABSTRACT
Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal
Infections (PANDAS) is defined as exacerbation of obsessive-compulsive disorder
and/or tic disorder after streptococcal infections. New accumulating evidence
suggests that PANDAS have some variants such as infantile-onset type, adultonset
type, myoclonic type, and dystonic type. In this report, we present the case of
a fifteen-year-old adolescent who had myoclonic symptoms in the shoulders and
was first treated for epilepsy and later, for conversion disorder until the diagnosis of
PANDAS was established. (Archives of Neuropsychiatry 2011; 48: 85-7)
Key words: PANDAS, myoclonus, late onset
Introduction
Streptococcal infections are very common in childhood.
Diseases attributed to streptococci are usually self-limited and
only a small part of them result in neurological and/or psychiatric
symptoms after infections (1). The most well-established among
these is Sydenham’s chorea, which has been long suspected to
have an autoimmune basis, specifically as a postinfectious
complication to group A β-hemolytic streptococcal (GABHS)
infection and rheumatic fever (RF) (2,3).
Pediatric autoimmune neuropsychiatric disorders associated
with streptococcal infections (PANDAS) is a term coined
by Swedo et al. to describe 50 patients with recurrent, acute
fulminant exacerbations of tics or obsessive-compulsive
symptoms that had a temporal association with GABHS infections.
ÖZET
PANDAS streptokoksik yang›dan sonra alevlenen obsesif kompulsif bozukluk
ve/veya tik bozuklu¤u olarak tan›mlanmaktad›r. Yeni biriken kan›tlar PANDAS’›n,
çocuklukta bafllayan, eriflkin bafllang›çl›, myoklonik tipte ve distonik tipte gibi
farkl› alt klinik tablolarla ortaya ç›kabilece¤ini bildirmektedir. Bu olgu sunumunda,
PANDAS teflhisi konulana kadar, önce epilepsi sonra konversiyon bozuklu¤una
yönelik tedavi alan, omuzlar›nda myoklonik semptomlar› olan on befl yafl›nda bir
ergen hastadan bahsedilecektir. (Nöropsikiyatri Arflivi 2011; 48: 85-7)
Anahtar kelimeler: PANDAS, myoklonus, geç bafllang›ç
Swedo et al. have also formulated 5 criteria of PANDAS: presence
of obsessive-compulsive disorder (OCD) and/or a tic
disorder, pediatric onset, episodic course of symptom severity,
association with GABHS infection, and neurological
abnormalities (4). Accumulating evidence suggests that
PANDAS has some variants such as infantile-onset type,
adult-onset type, myoclonic type, and dystonic type. Pavone
et al. also proposed some practical points for the diagnosis of
PANDAS variant: exclusion of tics/OCD criterion, importance of
GABHS infection and emphasis of the course of disease (5). In all
of the above-mentioned disorders, the symptoms vary, but
the etiology is almost the same. Autoimmune antibodies against
basal ganglia represent a common pathway in susceptible
persons. Genetic vulnerability seems to play an important role in
disease expression (6).
Address for Correspondence/Yaz›flma Adresi: Dr. Mehmet Fatih Ceylan, Kayseri E¤itim ve Araflt›rma Hastanesi, Çocuk ve Ergen Psikiyatrisi Klini¤i, Kayseri, Türkiye
Gsm: +90 505 393 02 79 E-mail: mehmetfceylan@yahoo.com Received/Gelifl tarihi: 20.04.2010 Accepted/Kabul tarihi: 14.08.2010
© Archives of Neuropsychiatry, published by Galenos Publishing. / © Nöropsikiyatri Arflivi Dergisi, Galenos Yay›nevi taraf›ndan bas›lm›flt›r.

86
In this report, we present the case of a fifteen-year-old
adolescent who had myoclonic symptoms in the shoulders
and was initially treated for epilepsy and later, for conversion
disorder until the diagnosis of PANDAS was established.
Case
Ceylan et al.
A Case of Myoclonic Symptoms After Streptococcal Infection: Possible PANDAS Variant
A fifteen-year-old male had been evaluated at the
emergency department with complaints of nausea and involuntary
movements of the shoulders a week after oropharyngeal infection
and high temperature. These symptoms had been considered
relevant to a diagnosis of encephalitis or an epileptic disorder.
Because the cerebrospinal fluid (CSF) findings had been
completely normal, encephalitis had been ruled out. Cranial MRI
and Electroencephalographic (EEG) assessment were without
any abnormalities. After initiating diazepam infusion, the
symptom of myoclonus had been controlled, hence, the patient
had been administered valproic acid at a dose of 500 mg per day
for the diagnosis of myoclonic epilepsy and had been discharged
after the disappearance of myoclonic seizures. Five days later,
the myoclonus had reappeared, so the dose of valproic acid had
been increased to 1000 mg per day, but with no response. In
spite of adding clonazepam 2 mg/day to the existing
pharmachotherapy, the myoclonus had persisted and showed
periodic pattern.
The patient was admitted to our outpatient clinic six months
after the onset of symptoms. Because there was no enough
response to antiepileptic treatment and sometimes the
symptoms were exacerbated by stressor factors, we requested
a consultation by the neurology department regarding the
possibility of conversion disorder. The last EEG assessment was
completely normal and the patient was prescribed sertraline 50
mg per day for the diagnosis of conversion disorder. The patient
has been followed up at the neurology and psychiatry
departments for ten months exhibiting the same symptom
severity.
At the final evaluation, it was found out that the symptoms
initiated after a high temperature and oropharyngeal infection
and also continued in an episodic pattern. Additionally, it was
understood that myoclonic symptoms followed skin lesions
determined to be pyogenic lesions. Skin swabs were taken for
culture by a dermatologist and were positive for streptococcal
infections, while the throat culture was negative.
Therefore, PANDAS myoclonic type has been suspected
and determination of Antistreptolysin-O (ASO) titer was
recommended. Because of the fact that the value of ASO was
high (345 IU/ml), all psychiatric and neurological treatments
were stopped and intramuscular (IM) penicillin treatment was
suggested for five days. Moreover, considering the probability of
acute RF, the rheumatology department was consulted, but this
diagnosis was not confirmed due to absence of other symptoms
of RF.
The myoclonus of the shoulders resolved completely with
penicillin therapy. Two months later, ASO titer was measured
again and was as high as the prior one. Thereupon, it was
decided that continuing penicillin prophylaxis (1.200.000 IU /
once every three weeks) was needed.
During the six-month follow-up period, the patient had no
complaints and continued his life in a healthy manner.
Discussion
Archives of Neuropsychiatry 2011; 48: 85-7
Nöropsikiyatri Arflivi 2011; 48: 85-7
In this report, we note that it is hard to recognize the
myoclonic variant of PANDAS in late-onset cases. According to
the diagnostic criteria of PANDAS, the symptom onset must
be between 3 years of age and puberty (7). The case presented
here does not meet the PANDAS criteria, but as there are cases
with even later onset, described as adult-onset variant of
PANDAS, we suggest that late onset can not be an exceptional
feature (8). Additionally, the sudden onset must be taken into
consideration as a supporting detail.
In the literature, there are few reports involving no tics and
obsessive-compulsive symptoms, but only myoclonus (9,10).
Pavone et al. noted that existence of OCD/tic disorders may not
be essential in PNADAS variant cases (5). The most common
autoimmune neuropsychiatric disorders associated with
streptococcal infections include Tourette’s syndrome,
Sydenham’s chorea, classic PANDAS and PANDAS variants (5).
In all these disorders, the symptoms vary, but the etiology is
almost the same. In each, it is hypothesized that antibodies
produced as part of an immune response to GABHS surface
proteins have antigenic similarities to central nervous system
proteins (molecular mimicry) in susceptible persons. Genetic
and familial factors may also play a role in the pathogenesis of
PANDAS. It has been found in RF that certain families have a
higher incidence of a monoclonal antibody, D8/17. Similarly,
patients with PANDAS have been shown to have similar
elevations of D8/17 (4).
The major clinical manifestations include tics and obsessivecompulsive
symptoms in PANDAS. This type of neuropsychiatric
symptoms is also observed in Sydenham’s chorea, which is the
only recognized neurological manifestation of RF. It is seen in
about 20% of patients with acute RF and most often occurs
weeks to months after group A streptococcal infection (11).
PANDAS patients do not present with classic choreiform
movements or other symptoms of RF, but only with tics and/or
obsessive-compulsive symptoms (12). Therefore, a detailed
anamnesis and exploration are necessary and caution should be
applied not to miss a RF.
Our case does not represent a classical PANDAS/PANDAS
variant case, but it is obvious that the myoclonic symptoms
were associated with streptococcal infection. Therefore, our
case points out the need for re-evaluation of PANDAS variant
criteria in the future.
Treatments including penicillin prophylaxis, intravenous
immunoglobulin, plasma exchange, tonsillectomy are useful in
this spectrum (1,13). Similarly, our case did not respond to
antiepileptic and antidepressant treatment, but responded to
penicillin.

Archives of Neuropsychiatry 2011; 48: 85-7
Nöropsikiyatri Arflivi 2011; 48: 85-7
It was observed that the first oropharyngeal infection and the
following pyogenic skin lesions were associated with the
myoclonic symptoms. It is known that PANDAS is related to
oropharyngeal infection by streptococci, but we also suggest
that streptococcus-associated skin lesions also may induce
PANDAS signs.
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A Case of Myoclonic Symptoms After Streptococcal Infection: Possible PANDAS Variant
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