Klumpke's Paralysis


Introduction

Klumpke paralysis is a neuropathy of the lower brachial plexus which may be resulted from a difficult delivery[1]. Usually the eighth cervical and first thoracic nerves are injured either before or after they have joined to form the lower trunk. This injury can cause a stretching (neuropraxia,), tearing (called “avulsion” when the tear is at the spine, and “rupture” when it is not), or scarring (neuroma) of the brachial plexus nerves. Most infants with Klumpke paralysis have the more mild form of injury (neuropraxia) and often recover within 6 months. 

The main mechanism of injury is hyper-abduction traction and depending on the intensity, cause signs and symptoms consistent with a neurological insult.

According to the the National Institute of Neural Disorders and Stroke (NINDS), there are four types of brachial plexus injuries that cause Klumpke’s :

  1. Avulsion, in which the nerve is severed from the spine.
  2. Rupture, in which tearing of the nerve occurs but not at the spine.
  3. Neuroma, in which the injured nerve has healed but can’t transmit nervous signals to the arm or hand muscles because scar tissue has formed and puts pressure on it.
  4. Neuropraxia or stretching, in which the nerve has suffered damage but is not torn.

Causes

Risk factors for Klumpke Paralysis are:

  1. large birth weight babies,
  2. maternal diabetes, 
  3. multiparity,
  4. difficult presentation,
  5. shoulder dystocia,
  6. forceps or vaccuum delivery,
  7. breech position,
  8. prolonged labor,
  9. previous child with obstetric palsy,
  10. intrauterine torticollis.
  11. Less common includes tumors (neuromas, rhabdoid tumors), intrauterine compression, hemangioma and exostosis of the first rib in the child.

Signs and Symptoms

Signs and symptoms:

- “Claw hand” is a classic presentation seen where the forearm is supinated and the wrist and fingers are flexed.

Other signs and Symptoms include:

  1. weakness and loss of movement of the arm and hand. Some babies experience drooping of the eyelid on the opposite side of the face as well. This symptom may also be referred to as Horner's syndrome. [2]
  2. decrease of sensation along the medial aspect of the distal upper extremity along the C8 and T1 dermatome.
  3. myotome findings that can range from decreasing muscular strength to muscular atrophy and positional deformity.
  4. Reflexes in the affected roots are absent.
  5. associated injuries clavicular and humerus fractures, torticollis, cephalohematoma, and facial nerve palsy.

- An infant with a nerve injury to the lower plexus (C8-T1) holds the arm supinated, with the elbow bent and the wrist extended.

Differential Diagnosis

  1. Erb's palsy; this injury affects the upper brachial plexus which will usually result in dermatome and myotome finds along the C5-C6 path[3],
  2. Distal nerve entrapment of the ulnar nerve at either the medial epicondyle of Guyon's tunnel- produces similar neurological findings as the more proximal Klumpke's. But there is no involvement of innervation proximal to the lesion, for example, pectoralis major involvement with true ulnar nerve entrapment[4].
  3. Thoracic outlet syndrome : TOS is a compression injury to the brachial plexus from a rudimentary rib, first rib, or the clavicle on the ipsilateral side, this could be post-traumatic, postural driven, and or genetic.It affects more than C8- T1 roots[5].
  4. Apical lung tumor
  5. Neurofibroma
  6. Disc herniation
  7. Shoulder impingement
  8. Clavicular or vertebral fracture
  9. Other

Management

Treatment of Klumpke’s injury in babies and children is heavily dependent on the severity and the classification of the injury.

The affected arm may be immobilized across the body for 7 to 10 days. For mild cases, gentle massage of the arm and range-of-motion exercises may be recommended.

For torn nerves (avulsion and rupture injuries), symptoms may improve with surgery.   

Surgical Options:

1. Surgery on the nerves (e.g., nerve grafts and neuroma excision).

2.Tendon transfers to help the muscles that are affected by nerve damage work better.

3.Muscle transfer, in which a less important muscle or tendon is removed from another part of the body and attached to the injured arm if the muscles there deteriorate

Physiotherapy Management

Physical therapy assists in keeping the muscles and joints’ range of motion normal. Physical therapy also keeps muscles and joints to work properly and prevents stiffness in joints such as the shoulder, elbow, or wrist. 

Physiotherapy majorly focuses on[6] :

  1. improving flexibility,
  2. range of motion,
  3. strength, and
  4. dexterity
  5. Pain control

The you tubes below show treatments of an infant with Klumpke Paralysis

[7]
[8]

References:

  1. Ulgen BO, Brumblay H, Yang LJ, Doyle SM, Chung KC. Augusta Déjerine-Klumpke, M.D. (1859-1927): a historical perspective on Klumpke's palsy. Neurosurgery. 2008 Aug;63(2):359-66; discussion 366-7
  2. Ruchelsman DE, Pettrone S, Price AE, Grossman J. Brachial Plexus Birth Palsy: An overview of early treatment options. Bulletin of the NYU Hospital for Joint Diseases. 2009;67(1):83-9.
  3. Jennett RJ, Tarby TJ, Krauss RL. Erb's palsy contrasted with Klumpke's and total palsy: different mechanisms are involved. Am. J. Obstet. Gynecol. 2002 Jun;186(6):1216-9; discussion 1219-20.
  4. Dy CJ, Mackinnon SE. Ulnar neuropathy: evaluation and management. Curr Rev Musculoskelet Med. 2016 Jun;9(2):178-84
  5. Freischlag J, Orion K. Understanding thoracic outlet syndrome. Scientifica (Cairo). 2014;2014:248163.
  6. Smania N, Berto G, La Marchina E, Melotti C, Midiri A, Roncari L, Zenorini A, Ianes P, Picelli A, Waldner A, Faccioli S, Gandolfi M. Rehabilitation of brachial plexus injuries in adults and children. Eur J Phys Rehabil Med. 2012 Sep;48(3):483-506.
  7. Anne and Robert H. Lurie. Brachial Plexus Palsy. Available from: https://www.youtube.com/watch?v=bvKrUEehkKc (last accessed 13.3.2019)
  8. Erb's Palsy with love. Available from: https://www.youtube.com/watch?v=V3KxSsZp_6c (last accessed 13.3.2019)