ALS Center

The ALS Center at LHMC

The ALS Center at Lahey Hospital & Medical Center is a multidisciplinary center devoted to the diagnosis and treatment of motor neuron diseases (MNDs) in adults, particularly amyotrophic lateral sclerosis (ALS – also known as Lou Gehrig’s disease).

Specialists from multiple departments including neurology, behavioral neurology, social work, respiratory, nutrition, and physical, speech and occupational therapy work together to provide patients with individualized, comprehensive care. Our multidisciplinary approach enables patients to maintain maximum neurological functioning in a supportive environment.

Overview of Motor Neuron Diseases

Motor neuron diseases, such as ALS, are a group of progressive neuromuscular diseases that destroy motor neurons, the cells that control muscle activity such as walking, breathing, speaking, and swallowing. When the muscles stop receiving signals from lower motor neurons, they weaken and shrink in size. They may also begin to spontaneously twitch. These twitches, also known as fasciculations, can be seen and felt beneath the skin. When lower motor neurons stop receiving signals from upper motor neurons in the spinal cord or brain, muscle stiffness (spasticity) and hyperactive reflexes can result. This can make voluntary movements slow and difficult. Over time, individuals with MNDs may lose their ability to walk or control other movements.

MNDs are classified according to whether the loss of function is genetically inherited or sporadic, as well as which neurons are affected.

Diagnosing ALS

ALS and other MNDs can vary dramatically in their initial presenting symptoms and physical manifestations. At our center, patients and caregivers meet with experienced neurologists and other specialists to establish a diagnosis. An ALS diagnosis requires comprehensive interviews with both patient and caregiver, neurological and mental status exams, and depending on the findings, additional studies to help narrow down possible causes. These studies may include brain and spinal cord imaging with magnetic resonance imaging (MRI), blood tests, genetic testing, electromyography/nerve conduction study (EMG/NCS), spinal fluid analysis, and muscle or nerve biopsy.

Goals of the ALS Center

The ALS Center aims to:

Provide ALS patients with optimal care and treatment, and support their families.
Maximize patient function, safety and comfort.
Educate patients and their family members about the disease and its treatment.
Facilitate patient participation in clinical trials.
Coordinate care with the patient’s primary care physician.

Treating ALS

Treating ALS seeks to maximize the patient’s quality of life. We focus on what our patients and their families tell us is important to them, utilizing a variety of medications to target specific symptoms such as muscle cramps, spasticity, pain, sleep disturbance, depression or agitation, excess saliva, and shortness of breath. Patients are seen regularly and may choose to participate in rehabilitation therapy with respiratory, speech, physical, and occupational therapists specially trained in ALS management.

Our team helps patients get three FDA-approved medications for ALS, including Riluzole, Radicava (Edaravone), and Relyvrio. Additionally, we are establishing clinical trials for ALS patients in collaboration with other institutions to advance ALS therapies.

We provide guidance and support for any concerns that may emerge, including personal safety, medical equipment, and home services.

Our social work team can guide patients and their caregivers as they begin to think about next steps, including establishing a power of attorney and health care proxy and how to begin considering financial planning and implications.

Teaching

The Neurophysiology – EMG Fellowship at Lahey Hospital and Medical Center is committed to training the next generation of clinical and academic experts in the diagnosis and management of neuromuscular diseases including ALS.

Publications on ALS

Quintana M., Saville B.R., Vestrucci M., Detry M.A., Chibnik L., Shefner J., Berry J.D., Chase M., Andrews J., Sherman A.V., Yu H., Drake K., Cudkowicz M., Paganoni S., Macklin E.A.; HEALEY ALS Platform Trial Study Group. Design and Statistical Innovations in a Platform Trial for Amyotrophic Lateral Sclerosis. Ann Neurol doi: 10.1002/ana.26714. Online ahead of print (2023). PMID: 37245090
Ghasemi M., Poulliot K., Daniello K.M., Silver B. Experience with Telemedicine in Neuromuscular Clinic During COVID-19 Pandemic. Acta Myologica 42(1): 14-23 (2023). PMID: 37091527
Mazzola M.A., Russell J.A. Neurology ethics at the end of life. Handb Clin Neurol 191: 235-257 (2023). PMID: 36599511
Fang T., Pacut P., Sun Y., Gao J., McNeilly B., Sivakumar S., Nascimento Andrade E.I., Bose A., Trombetta B., Ghasemi M. Clinical and genetic characteristics associated with ALS diagnostic timeline: a population-based study. Poster Presentation at The American Academy of Neurology (AAN) Annual Meeting, Boston, MA, USA (April 22-27, 2023).
Fang T., Je G., Pacut P., Keyhanian K., Gao J., Ghasemi M. Gene Therapy in Amyotrophic Lateral Sclerosis. Cells 11(13): 2066 (2022). PMID: 35805149
Paganoni S., Berry J.D., Quintana M., Macklin E., Saville B.R., Detry M.A., Chase M., Sherman A., Yu H., Drake K., Andrews J., Shefner J., Chibnik L., Vestrucci M., Cudkowicz M.E., for the HEALEY ALS Platform Trial Study Group. Adaptive Platform Trials to Transform ALS Therapy Development. Annals of Neurology 91(2): 165-175 (2022). PMID: 34935174
Je G., Keyhanian K., Ghasemi M. Overview of stem cells therapy in amyotrophic lateral sclerosis. Neurological Research 43(8): 616-632 (2021). PMID: 33632084
Ghasemi M., Keyhanian K., Douthwright C. Glial Cell Dysfunction in C9orf72-Related Amyotrophic Lateral Sclerosis and Frontotemporal Dementia. Cells 10(2): 249 (2021). PMID: 33525344
Russell J.A. Hastened death in veterans with amyotrophic lateral sclerosis. Muscle Nerve 63(6): 785-786 (2021). PMID: 33660291
Ghasemi M., Brown RH Jr. Genetics of Amyotrophic Lateral Sclerosis. Cold Spring Harb Perspect Med 8(5): a024125 (2018). PMID: 28270533
Felgoise S.H., Feinberg R., Stephens H.E., Barkhaus P., Boylan K., Caress J., Clawson L.L., Elman L., Goutman S.A., Mccluskey L., Russell J.A., Tiryaki E., Weiss M., Simmons Z. Amyotrophic lateral sclerosis-specific quality of life-short form (ALSSQOL-SF): A brief, reliable, and valid version of the ALSSQOL-R. Muscle Nerve 58(5): 646-654 (2018). PMID: 30028537
Ho D.T., Russell J.A. Mercury and motor neuron disease: Hooked on a hypothesis. Muscle Nerve (2018). PMID: 29443385
Peters O.M., Ghasemi M., Brown R.H. Jr. Emerging mechanisms of molecular pathology in ALS. J Clin Invest 125(5): 1767-1779 (2015). PMID: 25932674
Ho D.T., Ruthazer R., Russell J.A. Shoulder pain in amyotrophic lateral sclerosis. J Clin Neuromuscul Dis 13(1): 53-55 (2011). PMID: 22361626
Srinivasan J., Scala S., Jones H.R., Saleh F., Russell J.A. Inappropriate surgeries resulting from misdiagnosis of early amyotrophic lateral sclerosis. Muscle Nerve 34(3): 359-60 (2006). PMID: 16609978
Cudkowicz M.E., Shefner J.M., Schoenfeld D.A., Brown R.H. Jr, Johnson H., Qureshi M., Jacobs M., Rothstein J.D., Appel S.H., Pascuzzi R.M., Heiman-Patterson T.D., Donofrio P.D., David W.S., Russell J.A., Tandan R., Pioro E.P., Felice K.J., Rosenfeld J., Mandler R.N., Sachs G.M., Bradley W.G., Raynor E.M., Baquis G.D., Belsh J.M., Novella S., Goldstein J., Hulihan J.; Northeast ALS Consortium. A randomized, placebo-controlled trial of topiramate in amyotrophic lateral sclerosis. Neurology 61(4): 456-64 (2003). PMID: 12939417