Klumpke palsy

Klumpke palsy is a form of brachial plexus injury that primarily affects the lower roots, C8 and T1. It often presents with characteristic motor and sensory deficits in the hand and forearm. Understanding its anatomy, causes, and clinical features is essential for early diagnosis and effective management.

1. Anatomy and Neurophysiology

1.1 Brachial Plexus Overview

The brachial plexus is a network of nerves that originates from the spinal cord and innervates the upper limb. It is formed by the ventral rami of spinal nerves C5 to T1 and is organized into roots, trunks, divisions, cords, and terminal branches. The lower roots, C8 and T1, are primarily involved in Klumpke palsy.

1.2 Specific Nerves Involved in Klumpke Palsy

In Klumpke palsy, the C8 and T1 nerve roots are affected, which impacts the following nerves:

• Ulnar nerve – responsible for intrinsic hand muscle function and some forearm flexors
• Median nerve – partially affected, contributing to hand and finger flexion

1.3 Muscles and Functional Implications

Injury to the lower brachial plexus affects the muscles that control fine hand movements and flexion of the wrist and fingers. The primary muscles involved include:

• Intrinsic hand muscles, including interossei and lumbricals
• Flexor carpi ulnaris and part of flexor digitorum profundus

The loss of function results in characteristic hand deformities and weakness.

2. Etiology

2.1 Birth-Related Causes

Klumpke palsy commonly occurs during birth due to excessive traction on the infant’s arm. Contributing factors include:

• Difficult or prolonged labor
• Breech presentation
• Shoulder dystocia, where the shoulder becomes lodged behind the maternal pelvis

2.2 Traumatic Causes in Adults

In adults, Klumpke palsy can result from direct trauma or stretch injuries, such as:

• Falls with outstretched arms
• Motor vehicle accidents
• Sports-related injuries causing traction on the arm

2.3 Other Contributing Factors

Less common causes include:

• Tumors compressing the lower brachial plexus
• Cervical rib causing nerve entrapment
• Iatrogenic injuries during surgery or medical procedures

3. Pathophysiology

3.1 Nerve Injury Mechanisms

Klumpke palsy results from injury to the C8 and T1 nerve roots. The primary mechanisms include:

• Stretching of the nerves during excessive traction on the arm
• Avulsion, where the nerve root is torn from the spinal cord
• Compression from tumors, cervical ribs, or other anatomical anomalies

3.2 Effects on Muscle Innervation

Damage to the lower brachial plexus leads to paralysis or weakness of muscles innervated by C8 and T1. This includes:

• Intrinsic hand muscles, leading to impaired fine motor skills
• Flexor muscles of the forearm, resulting in weakened wrist and finger flexion

3.3 Sensory Impairment

Sensory deficits occur in areas supplied by the affected nerves. Patients may experience:

• Loss of sensation or numbness along the medial forearm
• Hypoesthesia or anesthesia of the ulnar side of the hand and fourth and fifth fingers

4. Clinical Presentation

4.1 Motor Deficits

Patients with Klumpke palsy typically present with a “claw hand” deformity due to paralysis of the intrinsic hand muscles. Additional motor deficits include:

• Weakness or paralysis of finger flexion and extension
• Reduced grip strength
• Difficulty performing fine motor tasks such as writing or buttoning

4.2 Sensory Deficits

Sensory impairment corresponds to the areas innervated by the C8 and T1 nerves. Common findings include:

• Numbness or tingling in the medial forearm and hand
• Loss of sensation along the ulnar side of the hand and fingers

4.3 Associated Findings

If the T1 nerve root involvement extends to sympathetic fibers, patients may develop Horner syndrome, characterized by:

• Ptosis, or drooping of the upper eyelid
• Miosis, or constricted pupil
• Anhidrosis, or reduced sweating on the affected side of the face

5. Diagnosis

5.1 Clinical Examination

Diagnosis of Klumpke palsy begins with a detailed physical examination. Key components include:

• Inspection for hand deformities, such as “claw hand”
• Assessment of motor function, including finger and wrist flexion and extension
• Evaluation of sensory function in the medial forearm and ulnar hand
• Testing reflexes, including biceps, triceps, and brachioradialis reflexes

5.2 Imaging Studies

Imaging may be used to identify structural causes of nerve injury or rule out associated conditions:

• MRI of the brachial plexus to detect nerve root avulsion or compression
• Ultrasound to visualize peripheral nerve injuries
• X-rays to identify bony abnormalities such as cervical ribs

5.3 Electrophysiological Studies

Electromyography (EMG) and nerve conduction studies are helpful for assessing the extent and severity of nerve damage. These studies can:

• Confirm involvement of C8 and T1 nerve roots
• Differentiate Klumpke palsy from other peripheral nerve injuries
• Guide prognosis and management planning

6. Differential Diagnosis

6.1 Other Brachial Plexus Injuries

It is important to distinguish Klumpke palsy from other brachial plexus injuries:

• Erb’s palsy, affecting C5 and C6 roots, usually presents with shoulder and elbow weakness
• Pan-plexus lesions, involving all roots C5 to T1, cause more extensive motor and sensory deficits

6.2 Peripheral Nerve Injuries

Other conditions to consider include isolated peripheral nerve lesions:

• Ulnar nerve injury, which may mimic hand weakness but lacks proximal involvement
• Median nerve injury, which affects thumb and index finger flexion without forearm involvement

6.3 Neuromuscular Disorders

Some neuromuscular disorders can present with similar symptoms and should be considered:

• Congenital muscular dystrophies affecting hand function
• Spinal cord lesions causing segmental weakness

7. Management

7.1 Non-Surgical Interventions

Initial management of Klumpke palsy often involves conservative approaches to preserve and improve function. These include:

• Physical therapy to maintain joint mobility and prevent contractures
• Occupational therapy to improve fine motor skills and adapt activities of daily living
• Splinting or orthotic devices to support the hand in a functional position

7.2 Surgical Interventions

Surgical management may be indicated for severe or persistent nerve injuries. Options include:

• Nerve grafting to repair avulsed or damaged nerve segments
• Nerve transfers to restore function in affected muscles
• Tendon transfers to improve grip strength and hand positioning

7.3 Supportive Care

Additional supportive measures focus on improving quality of life and preventing complications:

• Pain management using medications or nerve blocks
• Prevention of joint contractures through stretching exercises
• Patient education and family counseling on home care strategies

8. Prognosis and Complications

8.1 Recovery Rates

The prognosis of Klumpke palsy varies depending on the severity of the injury and timing of intervention. Partial injuries often show significant recovery within months, while complete avulsions may have poor functional outcomes without surgery.

8.2 Long-Term Functional Deficits

Persistent deficits may include:

• Limited hand and finger movement
• Weak grip strength
• Difficulty performing fine motor tasks

8.3 Potential Complications

Complications can arise from both the injury and prolonged immobilization:

• Joint contractures due to muscle imbalance
• Chronic pain or neuropathic pain syndromes
• Secondary musculoskeletal deformities from altered limb use

9. Prevention

9.1 Safe Obstetric Practices

Prevention of birth-related Klumpke palsy focuses on careful delivery techniques to minimize traction on the infant’s upper limbs. Strategies include:

• Proper management of shoulder dystocia using standardized maneuvers
• Consideration of cesarean delivery in high-risk presentations such as breech
• Training and preparedness of healthcare staff for difficult deliveries

9.2 Early Recognition and Management of Traumatic Injuries

Prompt identification of arm or brachial plexus trauma in adults can reduce the severity of Klumpke palsy. Measures include:

• Immediate evaluation following falls or motor vehicle accidents
• Immobilization and careful handling of the affected limb
• Referral to specialists for early intervention if nerve injury is suspected

9.3 Prenatal and Perinatal Care Considerations

Preventive measures during pregnancy and labor can lower the risk of neonatal Klumpke palsy:

• Monitoring fetal position and growth
• Planning delivery approach for high-risk pregnancies
• Minimizing excessive traction during labor

10. Future Directions and Research

10.1 Advances in Microsurgical Nerve Repair

Research in microsurgery is improving outcomes for patients with severe Klumpke palsy. Techniques include:

• Precision nerve grafting using autologous or synthetic grafts
• Advanced nerve transfer procedures to restore hand function
• Minimally invasive approaches to reduce surgical trauma

10.2 Neuroregenerative Therapies

Emerging therapies aim to enhance nerve regeneration and functional recovery, such as:

• Stem cell therapies targeting damaged nerve tissue
• Growth factor administration to promote axonal regeneration
• Electrical stimulation and neurorehabilitation techniques

10.3 Novel Rehabilitation Techniques

Innovations in rehabilitation are improving outcomes for patients with persistent deficits:

• Robotic-assisted therapy for fine motor skill improvement
• Virtual reality and computer-assisted occupational therapy
• Personalized rehabilitation programs based on electrophysiological assessment

References

1. Perkins J, Howard J. Brachial plexus birth palsy. Curr Opin Pediatr. 2008;20(1):55-60.
2. Pondaag W, Malessy MJ, Van Dijk JG, Thomeer RT. Natural history of obstetric brachial plexus palsy: a systematic review. Dev Med Child Neurol. 2004;46(9):138-144.
3. Gillies RA, Bae DS. Klumpke Palsy: Diagnosis and Management. Hand Clin. 2017;33(4):437-445.
4. El-Sayed AM. Obstetric brachial plexus injury: epidemiology, risk factors and prevention. Clin Obstet Gynecol. 2007;50(4):825-834.
5. Waters PM, Bae DS. Brachial plexus birth palsy: evaluation and management. J Am Acad Orthop Surg. 2008;16(9):547-558.
6. Kaye JJ, Coleman SS. Brachial plexus injuries in adults: Klumpke versus Erb lesions. Radiographics. 1990;10(4):687-705.
7. Hoeksma AF, Ter Steeg AM, Nelissen RG. Long-term functional outcome of obstetric brachial plexus injuries. Clin Rehabil. 2000;14(2):133-141.
8. Fiorentino NM, Gilbert A. Nerve transfers in Klumpke palsy. Hand Clin. 2019;35(2):199-210.
9. Shin AY, Spinner RJ. Adult brachial plexus injuries: operative and nonoperative management. J Am Acad Orthop Surg. 2007;15(11):687-697.
10. Pondaag W, Malessy MJ, Van Dijk JG. Surgical treatment of obstetric brachial plexus lesions. Clin Neurol Neurosurg. 2009;111(3):201-207.