Intracranial Involvement in Multiple Myeloma Presenting as a Cranial Nerve Palsy

Abstract

Multiple myeloma (MM) is characterized by the neoplastic proliferation of plasma cells producing a monoclonal immunoglobulin. Neurological complications in MM most frequently occur due to spinal cord compression by bony lesions, paraprotein-related neuropathy, hypercalcemia, hyperviscosity, or amyloidosis. Intracranial involvement is a rare complication of MM occurring in only 1% of patients. It can manifest as a solitary cerebral lesion, intra-parenchymal infiltration, or diffuse leptomeningeal disease. We present a case of a leptomeningeal myeloma in a 71-year-old woman with known relapsed MM presenting with a right sixth nerve palsy. Our patient was receiving spinal irradiation for a paraspinal plasmacytoma when she complained of double vision. Clinical exam revealed a right sixth nerve palsy. MRI revealed diffuse abnormal leptomeningeal thickening and enhancement typical for diffuse leptomeningeal infiltration. She was treated with whole brain irradiation and intrathecal methotrexate combined with a lenalidomide and dexamethasone chemotherapeutic regimen but unfortunately she passed away 5 weeks after onset of visual symptoms. MM involving the central nervous system (CNS) is a rare complication of MM and carries a poor prognosis with an average survival of 3 months. Due to its rarity, treatment of CNS MM is very heterogeneous. Thus case reporting is important to accumulate data on this rare presentation.

Radiation Therapy for Plasma Cell Disease of the Brain and Skull: Poor Palliation and Survival After Treatment for Central Nervous System Involvement

“…Notably, our results confirm already published data on the varied natural history of myeloma, with isolated lesions near neurologic structures associated with improved outcomes compared to patients with parenchymal lesions or LMD. 6,13,14 Furthermore, in our series, responses for patients with CN or INI were improved with doses above 15-20 Gy, and patients with anticipated longterm survival may benefit from these or higher doses. The presence of high-risk cytogenetics may also help guide decision making, as patients with poor molecular markers had worse clinical responses in our series.…”

A Rare Case of Epidural Myeloma Presenting as Recurrent Subdural Bleeding

“…Several risk factors have been identified for the development of CNS-MM. Deletions of chromosome 17p13.1 (p53) have been found in 89% of the CNS-MM patients [ 3 ]. In a retrospective study by Jurczyszyn et al, 39% of patients with CNS-MM had del(13q) and 23% had del(17p) [ 7 ].…”

“…CNS myeloma can cause heterogeneous neurological symptoms related to either spinal, cranial, or meningeal disease [ 8 ]. The symptoms are not exclusive to CNS infiltration by MM and can be found in other disease-related situations such as hypercalcemia, uremia, hyperviscosity syndrome, as well as side effects of drug therapy, and, in some cases, amyloid protein [ 3 ]. One such confounding factor that should be considered in myeloma patients is intracranial bleeding.…”

“…Neurological symptoms in MM most commonly occur due to complications such as spinal cord compressions by bone lesions, paraprotein-related neuropathy, hypercalcemia, hyperviscosity, or amyloidosis [ 3 ], while neurological symptoms due to CNS involvement of MM itself are rare and occur in one percent of patients [ 4 ]. The CNS involvement can manifest as a solitary cerebral lesion, intraparenchymal infiltration, diffuse leptomeningeal disease, or isolated dural involvement, while isolated dura mater involvement is extremely rare.…”

Schädelbasismetastasen mit Hirnnervenaffektionen

“…Schädelbasismetastasen mit Hirnnervendefiziten traten in unserer Analyse überwiegend als Metastasen von Prostatakarzinom, Mammakarzinom oder multiplem Myelom auf. In der Literatur dominieren Fallbeschreibungen von Patienten mit Prostatakarzinom [19,27,28,30] und multiplem Myelom [8,[22][23][24]. Beschreibungen von Patientinnen mit Mammakarzinom sind etwas seltener zu finden [5,6,18,19] Von den ersten neurologischen Symptomen bis zur Therapie vergingen im Mittel knapp 3 Monate.…”