Baylor University Medical Center Proceedings October 2017 - 455

Cavernous sinus syndrome
Rakul Nambiar, MD, and Sreejith G. Nair, DM

Cavernous sinus syndrome (CSS) is a condition characterized by multiple
cranial nerve palsies manifesting with ophthalmoplegia, ptosis, and facial sensory loss due to involvement of adjacent cranial nerves. Tumors,
trauma, and vascular, infectious, and noninfectious inflammatory disorders
have all been described as causes. Lymphomas have been reported to
involve the cavernous sinus, both as primary cavernous sinus lymphomas
or as secondary lesions. Here, we describe the case of a 63-year-old-man
with untreated chronic lymphocytic leukemia (CLL), diagnosed 4 years
earlier, who presented with CSS. Our patient underwent standard chemotherapy, but he succumbed to infection during the neutropenic period.

eoplastic B-cell inﬁltration in chronic lymphocytic leukemia (CLL) has been described in skin, lung, pleura,
kidney, and gastrointestinal tract tissues. However,
involvement of the central nervous system (CNS) is
very rare (1), and symptomatic CNS involvement in CLL is
known to be rarer (1). Reported cases of CNS involvement in
CLL have demonstrated a diverse and nonspeciﬁc spectrum of
symptoms: headaches, mental status changes, cerebellar signs,
cranial nerve abnormalities, and weakness of extremities (1).
Here we report an unusual case of a patient with untreated
CLL who presented with cavernous sinus syndrome (CSS).
To the best of our knowledge, CLL causing CSS has not been
reported previously.
CASE REPORT
A 63-year-old man presented with double vision. He ﬁrst
noticed diplopia 2 months prior to evaluation, stating it waxed
and waned in intensity. One month later, he had recurrent severe
diplopia, most prominent on leftward gaze, accompanied by
nausea, headache, and photophobia. His symptoms persisted
and progressed to include left-sided eyelid heaviness 1 month
after presentation. He had a 4-year history of asymptomatic Rai
stage I CLL (lymphocytosis with lymphadenopathy, without organomegaly or cytopenia). He was on regular follow-up during
the 4 years with blood counts, along with clinical examination
at 3-month intervals.
On examination, his Eastern Cooperative Oncology Group
performance score was 2. Neurological exam revealed left ptosis,
sluggish pupillary reﬂex, lateral gaze palsy, diminished medial
Proc (Bayl Univ Med Cent) 2017;30(4):455-456

gaze, and limited intorsion of the left eye. Visual acuities were
6/6 in the right eye and 6/9 in the left eye. Dilated fundus examination was normal. The left corneal reﬂex was absent, and
he also had hypoesthesia in the territory of the ﬁrst and second
divisions of the right trigeminal nerve. In addition, multiple
cervical lymph and axillary nodes were palpable, and the spleen
was palpated 2 cm below the left costal margin. The rest of the
neurological and physical examination was unremarkable.
The white blood cell count was 108,000/μL, and a peripheral blood smear revealed 60% atypical lymphocytes. His hemoglobin level was 11.5 g/dL and platelet count, 163,000/μL.
His lactate dehydrogenase level was 736 IU/L (normal range,
313-618 IU/L). Peripheral blood ﬂow cytometric analysis was
diagnostic of CLL. Bone marrow aspiration revealed 60% small
atypical lymphoid cells, and bone marrow biopsy showed interstitial and nodular inﬁltration by atypical lymphoid cells, having
clumped chromatin among normal hematopoietic elements.
Fluorescence in situ hybridization (deletion 11q, deletion 13q,
and deletion 17p) and conventional cytogenetic analysis of the
peripheral blood did not reveal any abnormalities. Magnetic
resonance imaging (MRI) of the patient's brain revealed an
asymmetric enhancing lesion in the left cavernous sinus, encasing the carotid artery and extending to the trigeminal cave
(Meckel's cave) (Figure). These ﬁndings were suggestive of neoplastic inﬁltration of the left cavernous sinus.
A diagnostic lumbar puncture was performed, and cerebrospinal ﬂuid (CSF) revealed a white blood cell count of 20
leukocytes/mm3 (lymphocytes, 80%; neutrophils, 20%), glucose of 80 mg/dL, and total protein of 54 mg/dL. Cytological
examination demonstrated the presence of small monomorphic
lymphocytes in the CSF suggestive of CLL cells. The patient
received high-dose methylprednisolone and was started on
ﬂudarabine (25 mg/m2/day intravenously for the ﬁrst 3 days),
cyclophosphamide (250 mg/m2/day intravenously for the ﬁrst
3 days), and rituximab (375 mg/m2/day intravenously on day
1) (FCR regimen). He improved symptomatically after steroids; however, his status worsened after chemotherapy and he
From Regional Cancer Centre, Trivandrum, India.
Corresponding author: Sreejith G. Nair, DM, Department of Medical Oncology,
Regional Cancer Centre, Trivandrum 695011, India (e-mail: sreejith.sg@gmail.
com).
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