AMD Masquerade Syndromes

Abstract

AIM: To report a case of rapid deterioration of primary intraocular lymphoma. METHOD(S): A case report RESULTS: A 73-year-old Chinese lady with no medical illness complained of seeing floaters in the left eye for one year. It was associated with deterioration in vision of the left eye. There was no eye pain, redness or prior trauma. She denied any neurological symptoms such as headache, nausea, vomiting or fits. Examination revealed right eye visual acuity of 6/24 correctable to 6/15 with pin-hole and left eye visual acuity of 6/45 not correctable with pin hole. Funduscopy revealed left vitreous clumps with vitritis and vitreous haemorrhage. A left vitrectomy and vitreos biopsy was performed. The results showed atypical lymphoid cells. Subsequently she developed worsening bilateral panuveitis with vitritis, with yellowish clumps and multifocal choroiditis at the posterior pole. A diagnosis of bilateral primary intraocular lymphoma was made. An initial CT Brain and ultrasound of the abdomen were negative for lymphoma. Review by the haematologic and neurologist did not reveal any significant features to suggest CNS or haematological involvement. She received ten courses of intravitreal methotrexate which she initially responded to. Later, she developed a few episodes of recurrences with the presence of new lesions. She was not started on chemotherapy or radiotherapy in view of the absence of neurological or systemic involvement. Unfortunately, the patient's general condition started to deteriorate five months after the diagnosis of primary intraocular lymphoma was made, with symptoms of lethargy, confusion and forgetfulness. Her vision also deteriorated to hand movements in both eyes. A whole body MRI was performed, which showed liver metastases. Three months later she became generally weak and subsequently became unresponsive. A CT brain showed subdural bleed, white matter oedema and gross midline shift, suggestive of brain metastases. She eventually succumbed to the disease. CONCLUSION(S): Primary intraocular lymphoma is a subset of primary central nervous system lymphoma (PCNSL). The diagnosis is difficult and requires a high index of suspicion. About 56% to 85% of patients who initially present with isolated primary intraocular lymphoma will develop cerebral lesions. Co-management with the neurologist is vital in the continuous management of these patients in view of the high rate of mortality.