Intravascular Malignant Lymphomatosis: A Cause of Subacute Dementia

Abstract

Intravascular malignant lymphomatosis (IML) is a rare disease characterized by proliferation of neoplastic cells of lymphoid origin within small blood vessels. The median survival of IML patients is only 6 months. Any organ can be affected, with or without clinical expression. Although skin lesions are classic, they are relatively uncommon (28%). Neurological symptomatology (which evolves over a few weeks) is the most common clinical expression (83%). Dementia is the most common neurological symptom that occurs in about half of the patients with central nervous system pathology, and is associated with poorer prognosis. The diagnosis is confirmed by histology but, except for lung, biopsies are not sensitive and are helpful only when performed in the symptomatic organs; furthermore, when associated with anesthesia, they can be followed by dramatic worsening of the patient''s condition. Elevated LDH is a good indicator of IML in patients with subacute neurological symptomatology, especially if associated with signs suggestive of other organ involvement.

Pulmonary Intravascular Lymphomatosis: Clinical, CT, and PET Findings, Correlation of CT and Pathologic Results, and Survival Outcome

“…IVL has been confused with other possible conditions including vasculitis, dementia, or infarction, occult neoplasm, and infection. When the lungs are involved, patients experience shortness of breath, hypoxemia, and, rarely, pulmonary hypertension, and the disease may simulate various lung diseases, particularly diffuse interstitial lung disease or a subacute or chronic infectious condition (6,7). Moreover, due to the rarity of this condition, ready diagnosis of the disease is difficult, and lung lesions in patients with IVL are often detected at autopsy (8).…”

Status Epilepticus as the Initial Presentation of Intravascular Lymphoma

“…Regarding the occurrence of seizures associated with IVL, epileptic seizures have been reported in 8.8–25% patients with IVL [4, 5, 6]. Epileptic seizures in patients with IVL reportedly occur at a more advanced stage in most cases [6, 7]; however, patients suffering from epileptic seizures at IVL onset have also been reported [4, 6, 8, 9]. Case reports and literature reviews have shown that epileptic seizures as the first manifestation of IVL are found in 3–4% patients with IVL [4, 6].…”

“…Epileptic seizures in patients with IVL reportedly occur at a more advanced stage in most cases [6, 7]; however, patients suffering from epileptic seizures at IVL onset have also been reported [4, 6, 8, 9]. Case reports and literature reviews have shown that epileptic seizures as the first manifestation of IVL are found in 3–4% patients with IVL [4, 6]. However, IVL initially presenting as SE has rarely been reported.…”

Intravascular lymphomatosis

“…One study reported that greater than 80% of patients diagnosed with intravascular lymphomatosis presented with neurological involvement, and half of those with CNS involvement presented with signs of dementia. 10 CNS involvement is associated with a poor prognosis. 10 Our patient typified the mean age of onset and neurological involvement, but lacked any skin findings.…”