Vol 41 # 3 September 2009 - Kma.org.kw

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KUWAIT MEDICAL JOURNAL September 2009
Case Report
Neurosarcoidosis as a First Presentation of
Systemic Sarcoidosis: Case Report
Sana Sawan, Majeda El-Saleh, Ibraheem Lasheen
Department of Medicine, Mubarak El-Kabeer Hospital, Kuwait
Kuwait Medical Journal 2009; 41 (3): 250-253
ABSTRACT
Neurosarcoidosis (NS) is a well-recognized
complication of systemic sarcoidosis, but it is
an uncommon first presentation of the disease.
Moreover, seizure disorder is a much less common
presentation of this subset. We report a case of a 30-
year-old male admitted with status epilepticus due
to NS. Magnetic resonance imaging (MRI) of the
brain demonstrated multiple nodular lesions. Chest
radiograph showed bilateral hilar lymphadenopathy
that proved by histopathological examination to
contain non-caseating granulomas (NCG). Other
causes of granulomatous lesions were excluded
by relevant investigations. Corticosteroids (CS)
markedly improved the clinical and radiological
findings. Seizures were well controlled by
antiepileptic drugs.
KEY WORDS: corticosteroids, magnetic resonance imaging, neurosarcoidosis, sarcoidosis, status epilepticus
INTRODUCTION
Sarcoidosis is an enigmatic multi-system
granulomatous disease of unknown etiology that
was first described in the nineteenth century [1] . It
affects people of all racial and ethnic groups and
occurs at all ages with a peak incidence at 20 to 39
years [2] .
Sarcoidosis usually involves the lungs in up to
90% of patients [1] . Neurosarcoidosis (NS) as a first
presentation of sarcoidosis with no prior evidence
of systemic involvement is extremely uncommon
and difficult to diagnose due to the long list of
differential diagnoses that range from tumors to
multiple sclerosis [3] . Only 5 to 10% of all patients
with systemic sarcoidosis manifest neurological
complications out of which about 50% were
previously diagnosed when the nervous system
gets involved [4,5] . Seizures are estimated to occur in
5 to 20% of NS patients [5] . Most reports indicate a
poor prognosis with severe progressing or relapsing
course when seizures complicate NS [5] . We describe
an unusual case of NS with status epilepticus as the
first presentation and illustrate the brain imaging,
histology results and response to treatment.
CASE REPORT
A 30-year-old male patient was brought to the
accident and emergency department of Mubarak
El-Kabeer hospital by ambulance in an unconscious
state after a sudden onset of three consecutive
tonic-clonic seizures witnessed by his wife. Few
minutes after arrival to the resuscitation room,
the patient sustained another fit witnessed by
the attending medical personnel. Accordingly, he
was sedated, intubated, mechanically ventilated,
loaded with diphenyl hydantion, and shifted to the
intensive care unit (ICU). He was pyrexial at 39 0 C,
tachycardiac at 120 beats / min, normotensive, with
no skin rash or palpable lymphadenopathy. Both
pupils were dilated but reactive to light and fundi
showed no papilledema. Except for leucocytosis,
his hemogram as well as biochemistry including
random blood sugar, calcium, magnesium,
renal and liver functions were all within normal
limits. The patient was immediately shifted to
radiology department for brain CT scan that
showed a one cm non-enhancing cystic lesion
with adjacent hyperdensity in the right temporal
lobe surrounded by vasogenic edema (Fig. 1). A
portable chest X-ray (CXR) showed bilateral hilar
lymphadenopathy (Fig. 2). A contrast enhanced
brain magnetic resonance imaging (MRI) done the
next day revealed a non-enhancing well-defined
one cm cystic lesion in the right hippocampus with
surrounding vasogenic edema and minimal mass
effect, as well as a nodular enhancement of the
suprasellar cistern and pituitary stalk. There was
also multiple enhancing parenchymal nodular areas
Address correspondence to:
Dr. Sana Sawan, PO Box 950, Hawally 32010, Kuwait. Tel:+ (965) 5349749, E-mail: sana_sawan2001@yahoo.com