Intrathecal baclofen Troubleshooting and Management

Michael Saulino, MD PhDMossRehab

Assistant Professor Thomas Jefferson University

Adjuvant Assistant ProfessorTemple University

CME Disclosures 1

• Speaker’s bureau for Jazz Pharmaceuticals

• Speaker’s bureau and clinical investigator for Medtronic, Inc

• Speaker’s bureau for Ipsen

• Consultant for SPR therapeutics and Myoscience

CME Disclosures 2

• Will discuss off label and investigational indications for medications and devices

• All activities are reviewed by Albert Einstein Healthcare Network’s conflict of interest committee

• Honoraria are paid directly to PMR department

Intrathecal baclofen therapy

Consider all factors to optimize therapy

Patients

• Given the divergence of disease conditions and presentations that be affected by ITB, it is crucial that clinicians counsel patients carefully regarding their expectations for this therapy

• Patients should be queried about their individual goals for treatment.

• They may indentify a wide variety of aspirations, anything from reduced caregiver burden to improved ambulatory capacity.

Patient factors

• Might be a problem with language

• “Spasms” and “Stiffness” can have multiple meanings

• Pain ≠ spasticity

Disease factorsVarieties of increased resistance to PROM

• Spasticity ITB approved• Dystonia ITB sensitive• Contracture not ITB sensitive• Heterotopic ossification• Ankylosis • Rigidity • Gegenhalten / paratonia

Diseases (approved indications)

• Spinal cord injury

• Multiple sclerosis

• Cerebral palsy

• Stroke

• Traumatic brain injury

Other illnesses potentially sensitive to ITB

• Non-traumatic spinal diseases: hereditary spastic paraparesis, transverse myelitis, ALS

• Dystonias

• Metabolic disorders: adrenoleukodystrophy

• Primary muscle diseases: tetanus, stiff person syndrome

Noxious stimuli

• Can increase or drive increase tone

• In a previously stable patient, increased tone can be the harbinger of a secondary influence

• Searching for this driving force can be a daunting task

Potential Noxious Stimuli

• Urinary: infection, stones, distention

• Dermatological: decubuti, surgical incisions

• Neurological: hydrocephalus, syringomyelia

• Pulmonary: pneumonia, coughing

• Medications: SSRIs

• Unusual: sunburn, bee stings, cancer

Agent

• Traditionally, effects of ITB have been related to two factors:– Catheter tip location

– Dosage administered

• Emerging data suggests that drug concentration / volume administered / flow rate can play a role in therapeutic effects.

Agent

• Does tolerance to ITB exist ?

• Reported as high as 30 % in the literature, although thorough investigation into other potential causes can be lacking

• Potentially addressed by bolus delivery or variable / complex dosing regimens

Catheter Micro-fractures

Dawes WJ, Drake JM, Fehlings D. Pediatr Neurosurg. 2003 Sep;39(3):144-8.

Agents

• Intrathecal Lioresal – FDA approved – Medtronic / Novartis

• Intrathecal Gablofen – FDA approved – CNS therapeutics

• Compounded baclofen – not FDA approved – state regulated, compounding pharmacies

Investigation Techniques for Catheter Problems

Plain radiography

Catheter dye study

CT myelogram

Nuclear medicine cisternogram

Magnetic resonance imaging

Device factors

• PumpPotential problems: pocket fill, battery failure, empty reservoir, programming errors, motor stall

• Catheter Potential problems: kinks, holes, blockages

CT myelogram after side port injection

• Remove 2-3 mls of fluid

• Inject 2-3 mls of isotonic contrast

• Patient is then immediately spiral scanned from 2 vertebral segments above the tip of the catheter, down through the pump in the abdomen.

CT myelogram dye study• Advantages: – Improved sensitivity compared to plain films

and fluoroscopy

– “Free” CT of abdomen, thorax, lumbar and thoracic spine

• Disadvantages: – Technical expertise

– Coordination with radiology

– Cannot undertake if fluid cannot be aspirated from side port

Normal study

Contrast loculated in the spinal canal

Contrast ventral to CSF suggesting subdural migration

Contrast extravasating into the subcutaneous tissues suggesting catheter breakage

Contrast filing of an

intrathecal granuloma

Clinicians

Intrathecal baclofen therapy

Consider all factors to optimize therapy