Recurrent Hodgkin’s Disease Presenting as a Clinically Isolated Cavernous Sinus Syndrome

Background. Hodgkin’s disease involving the central nervous system is uncommon and is usually seen in patients with uncontrolled systemic disease or those who have had multiple episodes of recurrent disease. Common symptoms of intracranial Hodgkin’s disease are motor and/or sensory deficits, headache, papilloedema, coma, and seizures. The rarity of Hodgkin’s disease presenting with intracranial involvement is marked, but patients presenting with cavernous sinus syndrome is even rarer. Despite its rarity, the presence of a cavernous sinus syndrome in a patient with a known history of Hodgkin’s disease warrants full utilization of modern diagnostic techniques in terms of investigation. Case Presentation. A 34-year-old woman, known with previous Hodgkin’s lymphoma and now in remission for the past 7 years, presented with signs and symptoms suggestive of a left cavernous sinus syndrome. She was otherwise systemically well with no other complaints. Extensive investigations revealed no obvious cause for the cavernous sinus syndrome. A CT chest revealed subclinical axillary lymphadenopathy, which on excisional biopsy confirmed recurrent Hodgkin’s disease. The patient’s sole clinical presentation of her recurrent disease was the cavernous sinus syndrome, with no other clinically obvious systemic signs or symptoms to suggest a relapse. This was treated with steroids, and clinical improvement was noted; she was referred to oncology for extensive chemotherapy. Conclusions. Whilst there is a paucity of literature around this topic, it must be remembered by the clinician that cavernous sinus syndrome may be the sole clinical presentation of recurrent Hodgkin’s disease. Even though it is reported that lymphomatous involvement in the brain usually occurs late in the disease process, this case is evidence that cavernous sinus syndrome may precede other systemic signs and symptoms. Recognising this possibility will ensure a rigorous search for Hodgkin’s disease, an early and effective diagnosis, and a better prognosis in affected patients.

Steroid responsive cavernous sinus syndrome due to Rosai-Dorfman disease: beyond Tolosa-Hunt syndrome – a case report

Background The term “Tolosa-Hunt syndrome” (THS) has been used to refer to painful ophthalmoplegia associated with nonspecific inflammation of the cavernous sinus and many processes can result in a similar clinical picture, including infectious, inflammatory and neoplastic diseases. Rosai-Dorfman disease (RDD) is a lymphoproliferative disorder that rarely affects the central nervous system. We report a case of isolated CNS Rosai-Dorfman disease involving the cavernous sinus and presenting as “Tolosa-Hunt syndrome”. Case presentation Our patient presented with horizontal diplopia due to impairment of cranial nerves III, IV and VI and a stabbing/throbbing headache predominantly in the left temporal and periorbitary regions. There was a nonspecific enlargement of the left cavernous sinus on MRI and the patient had a dramatic response to steroids. Biopsy of a frontal meningeal lesion was compatible with RDD. Conclusions We highlight the importance of including Rosai-Dorfman disease as a differential diagnosis in cavernous sinus syndrome and demonstrate a satisfactory long-term response to steroid treatment in this disease.

Anatomical Basis of Clinical Manifestations Seen in Cavernous Sinus Syndrome: A Narrative Review

Pathology involving the Cavernous Sinus (CS), generally referred to as Cavernous Sinus Syndrome (CSS), can arise from vascular disorders, neoplasms, infections, and non-infectious inflammatory disorders. An acute understanding of the CS and its regional anatomical structures is therefore pertinent in expounding the highly variable clinical manifestations seen in CSS as well as laying the groundwork for surgical intervention. Though the neuroanatomy of the cavernous sinus has been substantially chronicled in literature, their correlation to clinical signs has only been minimally described. This narrative review serves to address this knowledge gap and aims to comprehensively correlate the clinical manifestations of CSS with the relevant neuroanatomy, thereby allowing medical practitioners to better navigate the diagnostic quandary. We conducted a scoping review of the literature concerning CS anatomy and CSS, complied through MEDLINE/OVID and cross-referencing of articles on PubMed and Google Scholar with the keywords cavernous sinus, cavernous sinus syndrome, clinical signs/manifestations, neuroanatomy, CS pathology, Cavernous Sinus Thrombus (CST) and cavernous sinus neoplasms/tumours.

Lemierre’s syndrome with cavernous sinus thrombosis caused by dental infection

Lemierre’s syndrome is a rare multisystemic infection beginning in oropharynx commonly caused by oral anaerobic organisms and leading to internal jugular vein thrombosis with septic emboli. Here, we describe a 45-year-old woman with hypertension and unrecognised type 2 diabetes who presented to a community hospital with fever, double vision and septic shock. Examination showed neck pain aggravated by neck flexion, limited ocular movement of right lateral rectus, left medial rectus and left superior oblique and incomplete ptosis of the left eye. These symptoms were suggestive of bilateral cavernous sinus syndrome. CT of the brain showed bilateral proximal internal jugular vein and cavernous sinus thrombosis. CT angiography revealed septic emboli at both upper lungs. The patient had good improvement of neurological symptoms after dental extraction, intravenous antibiotic and anticoagulant.