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Benjamin Rix Brooks

Benjamin Rix Brooks, MD
Medical Director, Carolinas Neuromuscular/ALS - MDA Center
Department of Neurology
Associate Professor and Professor of Neurology, University of Wisconsin School of Medicine and Public Health

Prior Positions and Experience

1978 - 1982 NINDS Clinical Investigator Development Award and Assistant Professor of Neurology, Johns Hopkins University School of Medicine
1976 - 1978 NINDS Research Fellow, Neurovirology Laboratory, Johns Hopkins University School of Medicine
1974 - 1976 Clinical Associate and Senior Surgeon, US Public Health Service, Medical Neurology Branch, National Institute of Neurological and Communicative Disorders and Stroke, National Institutes of Health (Bethesda, Md.)
1972 - 1974 Residency, Neurology and Neuropathology, Massachusetts General Hospital (Boston, Mass.)
1970 - 1972 Residency, Internal Medicine, Boston City Hospital

Education

MD with Thesis: 1970, Harvard Medical School
AB: 1965, Harvard College (Cambridge, Mass.)

Research Interests

The different pathogenetic features of ALS/motor neuron disease may be attacked sequentially to develop empirical treatments for varied aspects of this condition. There has been success in developing riluzole, a disease-progression-modifying agent with multiple pharmacologic mechanisms of action that has a minimal to moderate effect on ALS progression in man and in animal models of motor neuron degeneration. Response to riluzole in man and animals seems to be age-dependent as well as a function of when treatment is initiated. The mechanisms for these findings are currently unknown.

More profound success has come from establishing that dextromethorphan, in combination with quinidine to provide stable high blood levels, can consistently control pseudobulbar affect which is prominent in ALS patients and those patients who have other diagnoses affecting the fronto-bulbo-cerebellar pathways. Treatments which control ALS symptoms need to be evaluated in combination with disease-progression-modifying-agents to see if there are more synergistic effects on survival and function.

Particular attention is necessary in addressing the special needs of ALS patients who develop bulbar symptoms which involve speech, swallowing and breathing difficulties either at the start of the disease or as the limb onset disease advances to the bulbar region. Symptomatic treatments need to be developed to minimize deficits in speech, chewing, swallowing and to minimize choking and aspiration leading to disease complications. Preventative methods need to be identified that will delay the occurrence of bulbar symptoms or slow their progression. Bulbar motor strength, particularly tongue strength and control, needs further study during the course of the disease in ALS patients to define where interventions may be introduced to minimize these symptoms.

Developing safe, cost-efficient and acceptable therapies to mitigate defined pathogenetic pathways in pre-clinical cell-based and animal models of motor neuron degeneration as well as ALS in humans requires analysis of these pathways individually at different phases of the disease process and collectively when the different disease processes may be active in different regions of the central nervous system and the peripheral neuromuscular system. ALS must be characterized carefully in each patient.

Lessons learned with respect to the limiting of the speed of progression, stabilizing the course of the disease transiently or consistently and even reversing disease progression will be tested in appropriate animal models to identify specific targets which might allow generalization to the wider ALS patient population and perhaps other motor neuron degenerations such as spinal muscular atrophy and dysimmune motor neuropathies.

Recent Publications

Gwinn K, Corriveau RA, Mitsumoto H, Bednarz K, Brown RH, Cudkowicz M, Gordon PH, Hardy J, Kasarskis EJ, Kaufmann P, Miller R, Sorenson E, Tandan R, Traynor BJ, Nash J, Sherman A, Mailman MD, Ostell J, Bruijn L, Cwik V, Rich SS, Singleton A, Refolo L, Andrews J, Zhang R, Conwit R, Keller MA; for The ALS Research Group [Brooks BR Steering Committee]. Amyotrophic lateral sclerosis: an emerging era of collaborative gene discovery. PLoS ONE 2007; 2: e1254. [PMID: 18060051]

Brooks BR. Involuntary emotional expression disorder: treating the untreated.
CNS Spectr 2007; 12: 23-27. [PMID: 17426672]

Konrad C, Jansen A, Henningsen H, Sommer J, Turski PA, Brooks BR, Knecht S.
Subcortical reorganization in amyotrophic lateral sclerosis.
Exp Brain Res 2006; 172: 361-369. [PMID: 16463149]

Cummings JL, Arciniegas DB, Brooks BR, Herndon RM, Lauterbach EC, Pioro EP, Robinson RG, Scharre DW, Schiffer RB, Weintraub D. Defining and diagnosing involuntary emotional expression disorder. CNS Spectr 2006; 11: 1-7. [PMID: 16816786]

Smith RA, Brooks BR. Treatment of pseudobulbar affect in ALS. Lancet Neurol 2005; 4: 270. [PMID: 15847840]

Current, Recent and Pending Grant Support

Grant Title: Safety and Efficacy of AVP-923 in PBA Patients With ALS or MS (STAR) (NCT00573443 07-AVR-123)
Funding Agency: Avanir Pharmaceuticals
Role:Steering Committee Member
Years: 2007 - present

Grant Title: Clinical Trial Ceftriaxone in Subjects With ALS (NCT00349622)
Funding Agency: National Institute of Neurological Disorders and Stroke (NINDS)
U01NS049640-02 through Northeast ALS (NEALS) Consortium Harvard Medical School, Massachusetts General Hospital
Role: Steering Committee Member
Years: 2007 - present

Grant Title: Tamoxifen Therapy in Amyotrophic Lateral Sclerosis (ALS) (NCT00214110)
Funding Agency: Muscular Dystrophy Association and National Institute of General Medical
Science General Clinical Research Center RR-03186
Role:Principal Investigator [Benjamin Rix Brooks, MD]
Years: 2005 - 2007

Grant Title: Collection of Blood Samples for DNA in Motor Neuron Disease (NCT00362362)
Funding Agency: National Institute of Neurological Disorders and Stroke (NINDS)
R01 NS049640-01 and NOT-NS-03-016 through Northeast ALS (NEALS) Consortium Harvard Medical School, Massachusetts General Hospital, Muscular Dystrophy Association and ALS Association
Role:ALS Research Group Steering Committee Member and site Principal Investigator
Years: 2005 - 2007

Grant Title: Safety/Efficacy of AVP-923 in the Treatment of Emotional Lability (Uncontrolled Crying & Laughing) in Patients With ALS (NCT00021697 99-AVR-102)
Funding Agency: Avanir Pharmaceuticals
Role:Steering Committee Member and Principal Investigator Years: 2001 - 2005

Benjamin Rix Brooks

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