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SSPE with chorioretinitis in a young female

Indonesian patient



Abstract: Subacute sclerosing panencephalitis is a rarely seen condition in Indonesia. The
disease report and distribution is unknown and many physicians are often misleaded to the
diagnosis of epilepsy. In this case report, we present a young female patient who had
myoclonus and mental deterioration with an EEG that supports the diagnosis of SSPE.
However, Lafora Body Disease should be considered due to the similarity of signs and
symptoms, unknown vaccination record and medical history of the patient prior to the onset
of myoclonus. Valproic acid 250mg BID was started orally for the myoclonus and a
reduction in frequencies was seen.

INTRODUCTION

SSPE is rare and progressive disease characterized by an onset of 5-15 years of age
with clinical manifestations of myoclonus and cognitive deterioration followed by death
within a few months in fulminant cases.
1
The deterioration experienced by SSPE patients are
commonly caused by an inflammation process as a result of fatal complication with a
persistent mutant measles virus acquired during early childhood.
2
In Indonesia, widespread
measles vaccination uptake differences between the rural and urban areas are
disproportionately low with approximately 0.9 million children missing measles vaccination
and a reported of 19,456 cases of measles infection amongst Indonesian children from the
low to medium socioeconomic family status.
3
Symptoms of SSPE ranges from changes in
behavior and mild mental deterioration such as memory loss to debilitating conditions such as
involuntary jerking movements, motor function disturbances, seizure or cortical blindness. As
the illness progresses, muscles become spastic due to ongoing central nervous system injury
followed by a vegetative state. SSPE patients commonly die as a result of fever, heart failure
or due to CNS dysfunction in maintaining the autonomic nervous system. Diagnosis of
confirmed SSPE is defined as clinical features of SSPE and the presence of measles-specific
IgG in CSF, regardless of titer. Probable SSPE is defined as clinical features of SSPE and
negative measles-specific IgG in CSF or when no lumbar puncture was performed.
CASE ILLUSTRATION

A 20 year old female was brought to the hospital because of her inability to stand,
walk and talk. The patient history reveals that the symptoms originated 11 months earlier. At
13 days postpartum, the patient had difficulty in dressing herself, blurred vision, slurred
speech and she was having difficulty in walking (dragging her feet). Patient was taken to an
accupuncture for treatment, but it had made the symptoms more exaggerated. On the second
month, her symptoms progressed to the point that she lost vision to both of her eyes, became
completely immobilized, anxious and had frequent crying spells. On the third month, the
patients right and left hands started to shake continuously except when she was asleep. At
this point, she was taken to a hospital in order to receive a head CT scan. Results from the
first hospital showed no abnormality on both hemispheres of the brain. Patient was then
discharged home with three different psychiatric medications and sleeping pills. Additionally,
she was given herbal medicine by a traditional healer, which was consumed for five
months. On the fourth month, patient started to suffer seizure attacks with flexion of both
arms and legs and spastic muscle tone. Patient was then taken to a physiotherapist where she
received heat treatments and exercises for two months. On the fifth month, the seizure attacks
disappeared, but she developed periodic jerking movements of a unilateral upper and lower
limb. There was no history of seizures before pregnancy, eclampsia or hemorrhage during
labor. Tetanus vaccination status is unknown. She gave birth at 34 weeks of gestation to a
viable 2,3kg male infant who is healthy and thriving presently. On the eleventh month,
patient was taken to the second hospital and a full investigation was performed. On physical
examination, she was conscious but unable to respond appropriately to questions being asked.
Vital signs were at normal range. Laboratory result showed normocytic normochromic
anemia (Hb 10,93 g/dL, Ht 34,84%). CT scan revealed subacute infarct to the right corona
radiata and right external capsule. EEG results showed spikes with multiple slow wave forms
on bilateral frontal lobes posterior region.

DISCUSSION

In this report, we present a case of progressive myoclonic epilepsy, mental decline,
visual disturbances that occurred to a young female patient 13 days postpartum. The patients
family reported routine antenatal care without any incident of illness or infection throughout
her pregnancy and vaccination records were unobtainable. She had an uncomplicated vaginal
birth at 34 weeks gestation to a viable, live infant weighing 2.3kg and was discharged home
three days later. The symptoms mentioned above started 13 days postpartum and gradually
worsened to complete immobilization, dissociated episodes of crying and smiling and
inability to communicate by 4 months after the onset of symptoms and continued to persist up
to this day.
On physical examination, her blood pressure, heart rate and systemic functions were
all within normal limits. Normal plasma electrolytes and hepatorenal function excluded
metabolic, renal and hepatic encephalopathy. Neurologic evaluation revealed poor
cooperation and orientation with occasional absurd behaviour. Direct and Indirect light
reflexes are intact and fundoscopy examination showed choreoretinitis on her left eye.(Figure
2) Upper and lower extremities exhibited spasticity and increased physiological reflexes.
Mini-mental state examination was not able to be conducted due to the uncooperative
condition of the patient. Laboratory investigations on routine blood, electrolytes, urinalysis
and biochemical testing are all within normal range, except that she is slightly anemic which
may be attributed to her deteriorated condition and decreased nutritional intake after delivery.
CT scan imaging taken revealed subacute infarct on the right corona radiata and external
capsule (Figure 1). An electroencephalography (EEG) evaluation revealed high voltage bursts
of polyphasic slow wave forms (Figure 3) which are typical patterns of SSPE.
In the differential diagnosis, Lafora Body Disease should be considered due to the
similar clinical manifestations in this patient. Lafora Body Disease is an autosomal recessive
hereditary disease that effects both sexes equally.
4
It is characterized by an onset of 6-20
years old with myoclonus followed by visual loss and cognitive decline in 1-2 years.
5
EEG
pattern consists of bursts of spike and wave which are generalized, multifocal and often
sensitive to illumination. The patients myoclonic frequency and generalized symptoms was
reduced with the use of valproic acid as supported by Footitt DR et al in their report on
Lafora Body disease management
4
, instead of oral isoprinosine plus interferon alpha that
would be used to repress further viral-induced damage of SSPE, thereby reducing the
symptoms severity, as suggested by Gokcil et al.
6,7
Diagnosis of Lafora Body is confirmed by
the demonstration of typical PAS positive spherical inclusion bodies in the brain, spinal cord,
skin, liver and skeletal muscle biopsies.
8,9
However, a biopsy was not performed because the
patient declined the procedure. The prognosis of both SSPE and Lafora Body Disease are
poor, with the range or life survival of 2-10 years prior to the onset of symptoms.
5,8,9,10

The patients disease onset, socioeconomic background, clinical manifestations and
EEG investigations are most consistent with subacute sclerosing panencephalitis.
1,2,3,10
SSPE
is an inflammatory disease in children and adolescents caused by persistent mutant measles
virus and are still common in developing and poor countries. Neuroimaging has a limited role
in the diagnosis of SSPE as early lesions will appear normal on CT scan. Magnetic
Resonance Imaging (MRI) is more sensitive in detecting white matter abnormalities, as early
lesions will dominantly involve the grey matter and subcortical white matter. The lesions are
asymmetrical and had a predilection for the posterior parts of cerebral hemispheres.
4
However, an MRI scan was not taken in this patient because of the familys objection. An
electroencephalogram (EEG) typical pattern of SSPE would consist of repetitive, polyphasic
slow wave complexes that recur every 4-15 seconds, which is present in our patient. An IgG
measles antibody titer is recommended to be taken, but the patient declined such procedure
thereby making this a probable case of SSPE.
In conclusion, the clinical manifestations of myoclonus, cognitive deterioration and
visual loss should be considered as SSPE and Lafora Body Disease
2,10
References
1). Comert.S, Vitrinel A, Gursu HA et al. Subacute sclerosing panencephalitis presenting as
acute disseminated encephalomyelitis. Indian Journal of Pediatrics 2006; 73(12):1119-21
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of Subacute Sclerosing Panencephalitis (SSPE): An analysis of twenty cases. Journal of
Dhaka Medical College 2008; 17 (2):72-77.
3). Fernandez RC, Awofeso N, Rammohan A. Determinants of apparent rural-urban
differentials in measles vaccination uptake in Indonesia. Rural Remote Health
2011;11(3):1702

4). Footott DR, Quinn N, Kocen RS, Oz B, Scaravilli F. Familial Lafora Body Disease of late
onset: report of four cases in one family and a review of the literature. J Neurol 1997; 244:
40-4.
5). Barreiro AF, Guillen JS, et al. A new case of late onset Lafora disease without generelised
seizures. J Neurology Neurosurgery Psychiatry 1999; 66: 114-6.
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factors and natural history. J of the Neurological Sciences 1996; 139 (2): 227-34.
7) Gokcil Z, Odabasi Z, Demirkaya S, et al. -Interferon and isoprinosine in adult-onset
subacute sclerosing panencephalitis. J of the Neurological Sciences1999;162:624.

8). Comoglu S. Et al. Diagnosis of Lafora Body Disease by Axillary Skin Biopsy. Turkish
Journal of Medical Science 2006; 36(4): 235-7.
9). Carpenter S, Karpeti G. Sweat gland duct cells in Lafora disease: Diagnosis by skin
biopsy. Neurology 1991; 31: 1564-8.
10). Buccoliero R, Palmeri S, Federico A. Ataxia, myoclonus and macular degeneration.
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Figure 1. CT Scan Sagittal Section














Figure 2. Funduscopy Examination