Klumpke Palsy - Causes, Classification, Diagnosis, and Treatment

Introduction:

Brachial plexus injury or brachial plexus lesion is an injury to the brachial plexus. It is a network of neurons that conduct signals from the spinal cord to the shoulder, arms, and hands. These nerves typically originate from the fifth (C5), sixth (C6), seventh (C7), and eighth (C8) cervical spinal nerves and the first thoracic (T1) spinal nerves. Injury to the brachial plexus can be either due to trauma or can be caused during the childbirth.

What Are the Causes of Brachial Plexus Injuries?

Brachial plexus injuries can be because of the following:

• Penetrating wounds.

• Missiles.

• Stab wounds.

• Injuries related to birth.

• Traction is applied to the plexus during falls.

• Vehicular accidents.

• Sports activities.

• Radiation.

What Are the Classifications of Brachial Plexus Injuries?

Brachial plexus injuries can be classified as

Leffert's Classification of Brachial Plexus Injury:

• Type I (Open).

• Type II (Closed).

• Type IIA Supraclavicular.

  • Preganglionic

    • Avulsion of nerve roots, usually from high-speed injuries.

    • Neuroma formation (neg Tinel's) or proximal stump is not present.

    • Pseudomeningocele and denervation of neck muscles are common.

    • Horner's Syndrome (ptosis, miosis, and anhydrosis).

  • Postganglionic

    • Roots remain intact.

    • Usually occurs from traction injuries.

    • Proximal stump and neuroma formation (pos Tinel's).

    • Deep dorsal neck muscles are intact, and pseudo meningocele will not develop.

• Type IIB Infraclavicular lesion

  • Usually develops branches from the trunks (supraclavicular).

  • The function is affected based on the trunk involved.

• Type III Radiation-induced.

• Type IV Obstetrics.

• Type IVA Erb's.

• Type IVB Klumpke.

Millesi Classification of Brachial Plexus Injury:

• Supraganglionic or preganglionic.

• Subganglionic or postganglionic.

• Trunk.

• Cord.

Classification-Based on Anatomy of the Location of Injury:

• Upper plexus palsy, or Erb's Palsy, involves C5 and C6 with or without C7. There is sensory loss over the lower part of the forearm and the lateral side of the deltoid. It involves the upper limb adducted and medially rotated with the elbow extended and pronated forearm. The deformity is called 'Policeman's tip hand' or 'Porter's tip hand.'

It is a more common form with a better prognosis.

• Lower plexus palsy, or Klumpke's Palsy, involves C8 and T1. It typically causes paralysis of all the intrinsic muscles of the hand, mainly lumbrical and interossei. This will result in loss of flexion of the metacarpophalangeal joints and loss of extension of the interphalangeal joints. The deformity results in claw hands due to hyperextension of the metacarpophalangeal joints and flexion of the interphalangeal joints. It is a rarer form with a worse prognosis.

• Total plexus lesion, which involves all nerve roots from C5 to T1.

What Are the Signs and Symptoms of Klumpke's Palsy?

The signs and symptoms of Klumpke palsy may range from mild to severe, depending upon the extent of nerve damage. They are listed as follows:

• Muscle weakness and atrophy.

• Minimum arm and hand movements.

• Sensory loss.

• Stiff joints.

• Claw Hands: It is a classic feature of Klumpke's palsy, in which the forearm lies flat, and the wrist and fingers are flexed. Cutaneous anesthesia and analgesia along the ulnar border of the forearm and hand.

• Horner's Syndrome: The symptoms of Horner's syndrome are:

  • Ptosis or drooping of the eyelid on one side of the face.

  • Miosis.

  • Anhidrosis.

  • Enophthalmos.

  • Loss of ciliospinal reflex.

• Vasomotor Changes:

  • Skin with sensory loss is warmer due to arteriolar dilatation.

  • Skin is dry as there is a loss of sympathetic activity.

• Trophic Changes:

  • Long-standing paralysis leads to scaly skin. In addition, the nails crack easily with the atrophied fingers.

What Are the Investigations to Be Carried Out?

Physical examination of all the nerve groups controlled by the brachial plexus to identify the specific site of the nerve injury and its severity. Few patients may also display specific signs that may help determine the injury's location, such as

• Narrowed eye pupils, droopy eyelids, and lack of ability to sweat are indicative of Horner's syndrome. It signifies that the injury is close to the spinal cord.

• Shooting, nerve-like pain on tapping along the affected nerves is indicative of Tinel's sign. It is a sign which suggests an injury that is far away from the spinal cord. Over time, if the location of the Tinel sign descends the arm towards the hand, it is a sign that the injury is healing itself.

• Radiograph of Cervical Spine: Fractures of lateral masses of cervical vertebrae are strongly associated with preganglionic injuries.

• Chest Radiograph: It may show first and second rib fractures or an elevated diaphragm, indicative of phrenic nerve paralysis and proximal injury to the upper plexus. Fractures of the scapula, clavicle, and humerus may indicate infraclavicular plexus injuries.

• Electromyography: The most important use of electromyography studies is for serial evaluation of injury to locate the signs of reinnervation. The appearance of prolonged, polyphasic, and low amplitude indicates reinnervation. In addition, a decreased number of fibrillation and sharp positive potentials is typically seen in denervated muscles.

• Computed Tomography (CT) Myelography: If a nerve injury has been suspected, a myelogram and subsequent CT scan should be obtained two to three months after the injury. It may be inaccurate after the injury as clotted blood may occlude the opening into the pseudomeningocele. Therefore, a delay of six to twelve weeks is recommended before a myelogram is ordered.

Advantages:

• It can detect partial root avulsion.

• It provides excellent visualization of the bony structures.

• It also provides a multiplanar reconstruction.

Disadvantages:

• It uses a very high radiation dose.

• In addition, it provides poor visualization of the lower brachial plexus due to bony artifacts.

Conventional Magnetic Resonance Imaging (MRI):

• MRI provides additional anatomic and physiologic information on injuries.

• Signal intensity changes in the spinal cord. Hyperintense areas on T2 weighted images suggest edema in the acute phase and myelomalacia in the chronic phase. In contrast, hypointense areas on T2 weighted images suggest hemosiderin deposition on account of hemorrhage.

• Enhancement of nerve roots is suggestive of functional impairment of nerve roots despite morphologic continuity.

• Enhancement of paraspinal muscles is suggestive of root avulsion injury.

What Is the Management of Klumpke's Palsy?

Closed brachial plexus injury requires a conservative approach that involves pain management with analgesics, opioids, and anticonvulsant drugs such as Gabapentin or Carbamazepine, maintaining the range of motions of the extremities, protecting the denervated dermatomes, and strengthening the remaining functional muscles. It does not require surgical exploration. However, open brachial plexus injury may require surgical interventions, typically in gunshot wounds.

What Are the Differential Diagnosis for Klumpke's Palsy?

Differential diagnoses include

• Erb's palsy.

• Apical lung tumor.

• Thoracic outlet syndrome.

• Disc herniation.

• Neurofibroma.

• Shoulder impingement.

• Clavicular or vertebral fracture.

Conclusion:

Injury to the brachial plexus can be either due to trauma or obstetrics caused during childbirth. Brachial plexus injuries are difficult to manage and usually improve with time without specific interventions. For example, managing Klumpke's palsy typically involves increasing the range of motion of the extremities, controlling pain, and preventing muscle atrophy.