Feb. 24, 2023
Leptomeningeal inflammation can indicate many conditions, making precise diagnosis of the underlying disease difficult. Misdiagnosis can lead to treatment that harms patients.
Mayo Clinic has launched a multidisciplinary group focused on improving diagnosis for patients with leptomeningeal diseases.
"It's an area of neurology that's very often overlooked," says Ivan (Darin) D. Carabenciov, M.D., a neurologist at Mayo Clinic in Rochester, Minnesota. "Many dangerous conditions show up in the leptomeninges. But patients are many times misdiagnosed, or there's a profound delay in the diagnosis, due to a lack of familiarity with these conditions and the unavailability of appropriate examinations."
As a high-volume center, Mayo Clinic has experience managing individuals with leptomeningeal inflammation and the sophisticated technology required to evaluate them.
"The detection of leptomeningeal disease with imaging is challenging," says Karl N. Krecke, M.D., a neuroradiologist at Mayo Clinic's campus in Minnesota. "Effective diagnosis requires the critical use of contrast materials and expertise in the subtleties of leptomeningeal inflammatory disease."
Evaluations are tailored to individual patients' needs. "When initial examinations don't provide the clues needed for a clear diagnosis, patients can benefit from referral to a high-volume center with experience in leptomeningeal diseases," Dr. Carabenciov says.
Sophisticated imaging before treatment
Leptomeningeal inflammation is associated with cancer, infections and autoimmune disorders such as sarcoidosis. As a result, patients can initially present with a variety of symptoms, including double vision, numbness in the face, positional headaches and syncope.
"The variability of symptoms is another reason why diagnosis can be very difficult," Dr. Carabenciov says.
Syncope is particularly concerning. "It can occur many times during the day, and it's a signature of increasing intracranial pressure," Dr. Carabenciov says. "Intracranial pressure spikes are underrecognized among clinicians, and these spikes are dangerous because occasionally they can generate arrhythmia."
Diagnosis also can be clouded by previous misdiagnosis and treatment. "Clinicians might immediately prescribe steroids for patients with leptomeningeal inflammation. But steroids can affect test results and end up significantly delaying the ultimate diagnosis," Dr. Carabenciov says.
Mayo Clinic's multidisciplinary leptomeningeal team thoroughly evaluates each patient before initiating treatment. Sophisticated imaging is a critical step.
"It is uncommon for early stages of leptomeningeal disease — inflammatory or neoplastic — to produce masses of cells that are obvious on CT or MRI exam," Dr. Krecke says. "MRI of the brain or spinal canal with intravenous contrast material is the key to early detection. Techniques such as T2-fluid-attenuated inversion recovery sequences with contrast enhancement can heighten sensitivity to these unusual diseases. Positron-emission tomography examinations can also be useful to assess disease manifestations outside the neuroaxis."
Once clear diagnoses are reached, patients have subspecialized treatment from Mayo Clinic neurologists and neurosurgeons. "Our strong core group has expertise in multiple fields, including neuro-oncology and autoimmune neurology," Dr. Carabenciov says.
To further improve the management of leptomeningeal inflammation, Mayo Clinic specialists are developing a diagnostic algorithm. "The goal is to make the patient work-up as efficient as possible without casting too wide a net," Dr. Carabenciov says.
Mayo Clinic researchers are studying the records of individuals with leptomeningeal inflammation who had biopsies to determine what information was gained and whether less invasive testing might yield similar information.
"Our hope is to develop even greater expertise in the diagnosis and treatment of patients with leptomeningeal inflammation," Dr. Carabenciov says.
For more information
Refer a patient to Mayo Clinic.