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3. Steroid-Responsive Parkinsonism and Encephalopathy: A Case Report

Author

Tofade, Toluwalase Oluwakemi, Harrold, G. Kyle, Laud, Arjun, Wills, Anne-Marie, and Venna, Nagagopal

Abstract

We describe a patient who presented with rapidly progressive parkinsonism and encephalopathy and was diagnosed with seronegative autoimmune encephalitis (AE). Subacute parkinsonism as a manifestation of seronegative AE is uncommon with only a handful of similar cases published in literature. A 71-year-old man presented with severe flu like symptoms, rapidly progressive cognitive decline and was found to have parkinsonian features on examination. Initial brain magnetic resonance imaging (MRI) was unremarkable however, cerebrospinal fluid (CSF) analysis revealed a lymphocytic pleocytosis and elevated protein level. Thorough searches for neural antibodies and infectious pathogens were negative. His symptoms fluctuated initially but markedly improved within days of starting prednisone and dramatically worsened after prednisone was tapered off. His CSF pleocytosis also improved on prednisone. Relapses again resolved with resumption of prednisone. The scope of autoimmune neurology Is constantly evolving, and physicians should be aware of the diverse and heterogenous clinical presentations of autoimmune encephalitis. We aim to emphasize the importance of ruling out autoimmune encephalitis in patients presenting with acute or subacute parkinsonism. This case additionally reinforces that negative antibody tests do not exclude the diagnosis of AE.

Published
2024
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6. Contributors

Author

Baker, Jessica M., Benge, Elizabeth, Berkowitz, Aaron L., Bhattacharyya, Shamik, Chen, Anderson, Daffner, Kirk R., Darsey, Melissa, Doughty, Christopher T., Erkkinen, Michael, Feske, Steven, Gale, Seth A., Hall, Kathryn E., Harrold, G. Kyle, Hayes, Michael T., Holmes, Manisha G., Howard, Danielle M., Jahan, Sultana, Knief, Alexandra, Lee, Eudocia Q., Lee, Thomas C., Manzano, Giovanna S., Marciano, Sadie P., Milligan, Tracey A., Moshe-Lilie, Orly, Mullally, William J., Novak, Peter, O'Neal, Mary Angela, Pavlova, Milena, Prasad, Sashank, Renthal, William, Reynolds, Gretchen, Rizzoli, Paul B., Rubin, Daniel B., Sadjadi, Reza, Salajegheh, Mohammad Kian, Samuel, Ananya Ruth, Schneider, Andrew G., Stanley, Michael P.H., Thakore-James, Manisha, Vgontzas, Angeliki, Wang, Victor C., Whitman, Gregory T., Willment, Kim C., Youssef, Gilbert, and Zurawski, Jonathan

Published
2025
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8. Contributors

Author

Abrams, Annah N., Alpay, Menekse, Alpert, Jonathan E., Baig, Mirza, Bains, Ashika, Baker, Amanda Waters, Beach, Scott R., Beck, BJ, Beckwith, Noor, Beresin, Eugene V., Bird, Suzanne A., Blais, Mark A., Bomm, Marie D., Bosson, Rahel, Brendel, Rebecca Weintraub, Bui, Eric, Camprodon, Joan A., Caplan, Jason P., Cassano, Paolo, Celano, Christopher M., Charoenpong, Prangthip, Chemali, Zeina, Chen, Justin A., Chopra, Amit, Cohen, Jonah N., Cohen, Lee S., Colvin, Mary Kathryn, Conteh, Nkechi T., Cremens, M. Cornelia, Cusin, Cristina, Donovan, Abigail L., Dougherty, Darin D., Eddy, Kamryn T., Evanoff, Anastasia B., Evins, A. Eden, Fava, Maurizio, Fernandez-Robles, Carlos G., Finn, Christine T., Flaherty, Alice W., Frates, Elizabeth Pegg, Freeman, Marlene P., Freudenreich, Oliver, Fricchione, Gregory L., Gatchel, Jennifer R., Gelaye, Bizu, Georgiopoulos, Anna M., Ghaznavi, Sharmin, Gholipour, Taha, Gold, Alexandra K., Gordon, Christopher D., Greenberg, Donna B., Greer, Joseph A., Groves, James E., Grunberg, Victoria A., Harrold, G. Kyle, Hazen, Eric P., Henry, Michael E., Hogan, Charlotte S., Hooker, Julia E., Huffman, Jeffery C., Irwin, Kelly Edwards, Ivkovic, Ana, Jacobs, Jamie M., Jain, Felipe A., Jankauskaite, Greta, Januzzi, James L., Jr., Jochimsen, Kate N., Kamali, Masoud, Katz, Tamar C., Keuroghlian, Alex S., Kim, Hyun-Hee, Kim, Youngjung Rachel, King IV, Franklin, Koh, Katherine A., Kohrman, Samuel I., Kontos, Nicholas, Lim, Carol, Linnoila, Jenny J., Luccarelli, James, Madva, Elizabeth N., Manglani, Heena R., McCoy, Thomas H., Jr., McDermott, Katherine A., Mischoulon, David, Morfin Rodriguez, Alejandra Elizabeth, H. Nejad, Shamim, Newhouse, Amy L., Nierenberg, Andrew A., Nisavic, Mladen, Nonacs, Ruta, Ostacher, Michael J., Papakostas, George I., Park, Elyse R., Peay, Celeste, Pederson, Aderonke Bamgbose, Perlis, Roy H., Peters, Amy T., Prager, Laura M., Praschan, Nathan, Prince, Jefferson B., Probert, Julia M., Prom, Maria C., Punko, Diana, Raviola, Giuseppe J., Robinson, Ellen M., Roffman, Joshua L., Rubin, David H., Rush, Christina L., Schlozman, Steven C., Schouten, Ronald, Shafer, Linda C., Jennifer Sheets, Sher, Yelizaveta, Sherman, Janet C., Smith, Felicia A., Sorg, Emily M., Stern, Theodore A., Stoler, Joan M., Sylvia, Louisa G., Taylor, John B., Thom, Robyn P., Tillman, Emma M., Trinh, Nhi-Ha, Vranceanu, Ana-Maria, Wang, Betty, Watkins, Khadijah Booth, Weinberg, Marc S., Weinstein, Sylvie J., Wiechers, Ilse R., Winkelman, John W., Yeung, Albert S., and Zambrano, Juliana

Published
2025
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9. Mogamulizumab for Treatment of Human T-lymphotropic Virus Type 1-Associated Myelopathy/Tropical Spastic Paraparesis: A Single-Center US-based Series.

Author

Meyerowitz, Eric A, Mukerji, Shibani S, Harrold, G Kyle, Erdil, Rachel M, Chen, Steven T, Rudmann, Emily A, Tsibris, Athe, Venna, Nagagopal, and Robbins, Gregory K

Subjects
THERAPEUTIC use of monoclonal antibodies, RETROVIRUSES, SPINAL cord diseases, CELL receptors, MONOCLONAL antibodies, EXANTHEMA, MYELITIS
Abstract

Background Human T-lymphotropic virus type 1 (HTLV-1)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) is a chronic neurological condition characterized by progressive myelopathic symptoms including spasticity, pain, weakness, and urinary symptoms, without proven treatments. Mogamulizumab (MOG) is a monoclonal antibody that binds CCR4 and leads to the clearance of HTLV-1-infected CCR4+ cells. A phase 1-2a study in Japan evaluated MOG for the treatment of HAM/TSP and reported decreases in HTLV-1 proviral load and neuroinflammatory markers, with clinical improvement in some participants. Methods We administered MOG 0.1 mg/kg every 8 weeks to individuals with HAM/TSP as a compassionate and palliative treatment. Patients who received MOG had (1) a positive peripheral HTLV-1 antibody, (2) progressive myelopathic symptoms, and (3) a diagnosis of HAM/TSP. Results Four female patients, ages 45–68, received MOG (range, 2–6 infusions) between 1 November 2019 and 30 November 2022. Two patients with <3 years of symptoms had milder disease, with Osame scores <4. The other 2, with >7 years of symptoms, had Osame scores >5. One patient, with 6 total treatments, received dose-reduced MOG after she developed a rash at the initial dose. The 2 patients with milder baseline disease reported symptomatic improvement and saw reductions in Osame and/or modified Ashworth scale scores during follow-up. The other 2 patients showed no improvement. All 4 developed rashes after receiving MOG—a treatment-limiting event in some cases. Conclusions Clinical trials are needed including diverse patient populations to assess the potential role of MOG for HAM/TSP. Our findings may help inform the development of these trials. [ABSTRACT FROM AUTHOR]

Published
2023
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10. Susceptibility-weighted imaging reveals cerebral microvascular injury in severe COVID-19

Author

Conklin, John, Frosch, Matthew P., Mukerji, Shibani S., Rapalino, Otto, Maher, Mary D., Schaefer, Pamela W., Lev, Michael H., Gonzalez, R.G., Das, Sudeshna, Champion, Samantha N., Magdamo, Colin, Sen, Pritha, Harrold, G. Kyle, Alabsi, Haitham, Normandin, Erica, Shaw, Bennett, Lemieux, Jacob E., Sabeti, Pardis C., Branda, John A., Brown, Emery N., Westover, M. Brandon, Huang, Susie Y., and Edlow, Brian L.

Published
2021
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11. Susceptibility-weighted imaging reveals cerebral microvascular injury in severe COVID-19

Author

Massachusetts Institute of Technology. Institute for Medical Engineering & Science, Massachusetts Institute of Technology. Department of Brain and Cognitive Sciences, Conklin, John, Frosch, Matthew, Mukerji, Shibani S., Rapalino, Otto, Maher, Mary D., Schaefer, Pamela W., Lev, Michael H., Gonzalez, R.G., Das, Sudeshna, Champion, Samantha N., Magdamo, Colin, Sen, Pritha, Harrold, G. Kyle, Alabsi, Haitham, Normandin, Erica, Shaw, Bennett, Lemieux, Jacob E., Sabeti, Pardis, Branda, John A., Brown, Emery Neal, Westover, M Brandon, Huang, Susie Y., Edlow, Brian L., Massachusetts Institute of Technology. Institute for Medical Engineering & Science, Massachusetts Institute of Technology. Department of Brain and Cognitive Sciences, Conklin, John, Frosch, Matthew, Mukerji, Shibani S., Rapalino, Otto, Maher, Mary D., Schaefer, Pamela W., Lev, Michael H., Gonzalez, R.G., Das, Sudeshna, Champion, Samantha N., Magdamo, Colin, Sen, Pritha, Harrold, G. Kyle, Alabsi, Haitham, Normandin, Erica, Shaw, Bennett, Lemieux, Jacob E., Sabeti, Pardis, Branda, John A., Brown, Emery Neal, Westover, M Brandon, Huang, Susie Y., and Edlow, Brian L.

Abstract

We evaluated the incidence, distribution, and histopathologic correlates of microvascular brain lesions in patients with severe COVID-19. Sixteen consecutive patients admitted to the intensive care unit with severe COVID-19 undergoing brain MRI for evaluation of coma or neurologic deficits were retrospectively identified. Eleven patients had punctate susceptibility-weighted imaging (SWI) lesions in the subcortical and deep white matter, eight patients had >10 SWI lesions, and four patients had lesions involving the corpus callosum. The distribution of SWI lesions was similar to that seen in patients with hypoxic respiratory failure, sepsis, and disseminated intravascular coagulation. Brain autopsy in one patient revealed that SWI lesions corresponded to widespread microvascular injury, characterized by perivascular and parenchymal petechial hemorrhages and microscopic ischemic lesions. Collectively, these radiologic and histopathologic findings add to growing evidence that patients with severe COVID-19 are at risk for multifocal microvascular hemorrhagic and ischemic lesions in the subcortical and deep white matter., National Institute of Neurological Disorders and Stroke (Grants R21NS109627, R21AG067562, RF1NS115268), NIH Director’s Office (Grant DP2HD101400), NIH National Institute of Mental Health (Grant K23MH115812), NIH National Institutes of Allergy and Infectious Diseases (Grant 2U19AI110818)

Published
2021

13. Cerebral Microvascular Injury in Severe COVID-19

Author

Conklin, John, primary, Frosch, Matthew P., additional, Mukerji, Shibani, additional, Rapalino, Otto, additional, Maher, Mary D., additional, Schaefer, Pamela W., additional, Lev, Michael H., additional, Gonzalez, R. G., additional, Das, Sudeshna, additional, Champion, Samantha N., additional, Magdamo, Colin, additional, Sen, Pritha, additional, Harrold, G. Kyle, additional, Alabsi, Haitham, additional, Normandin, Erica, additional, Shaw, Bennett, additional, Lemieux, Jacob E., additional, Sabeti, Pardis C., additional, Branda, John A., additional, Brown, Emery N., additional, Westover, M. Brandon, additional, Huang, Susie Y., additional, and Edlow, Brian L., additional

Published
2020
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