(Kiosk 5) Successful Treatment of Spasticity via Intrathecal Baclofen Pump Therapy in a Patient with ALS

Case Diagnosis: Amyotrophic lateral sclerosis (ALS)

Case Description or Program Description: A 41 year old female with a past medical history of Amyotrophic lateral sclerosis (ALS) (HCC) presenting to clinic after Baclofen pump implantation for management of spasticity. She was first diagnosed 2 years prior after noticing a gradual onset of progressively worsening symptoms. Initially, she experienced had left-hand weakness followed by slowing of her speech. Her gait became slower and stiffer. A month later, she noticed right hand weakness affecting her dexterity and causing her difficulty while doing ADLs. Months later her disease process progressed involving bilateral lower extremity weakness (L>R) and dysphagia. Her limitations with swallowing food led to a significant weight loss and nutritional decline. Within months, she developed dyspnea and orthopnea as well and was evaluated worked up by neurology. She was referred to pain clinic for spasticity management. She underwent successful baclofen trial and underwent implantation of intrathecal baclofen pump implant six months later. Since implantation, her pain has improved and care giver burden has decreased significantly. Patient is currently taking Riluzole for her disease progression, gabapentin for neuropathic pain, and magnesium for muscle cramping.

Setting: Outpatient Clinic

Assessment/Results: Patient was weaned off of orals and successfully managed via intrathecal baclofen pump for her spasticity without major complications.

Discussion (relevance): ALS is a degenerative motor neuron disease characterized by severe movement disorders. The disease progressively results in respiratory insufficiency, spasticity and painful muscle which limits quality of life. ALS is a progressive neurodegenerative disorder. Baclofen, an agonist of γ-amino butyric acid, is one of the most effective drugs in the treatment of spastic movement disorders. However, higher oral dosages required for sufficient spasticity control are related to intolerable central side effects3. The use of intrathecal baclofen in treating amyotrophic lateral sclerosis has not been commonly studied.

Conclusions: This report highlights a rare case of successful spasticity management via intrathecal Baclofen pump implantation in a patient with ALS.