Chapter 54

#### High-Yield Facts

• Upper motor neuron diseases usually present with asymmetrical weakness contralateral to the lesion. Lower motor neuron diseases present with symmetrical weakness that can be isolated to specific muscle groups.

• Involvement of bulbar muscles is manifested by cranial nerve findings, facial muscle weakness, and chewing or swallowing difficulties. Bulbar involvement can occur in both upper and lower motor neuron disorders.

• Neuropathies are disorders of nerves and tend to cause distal muscle weakness, hypesthesias or paresthesias, and decreased reflexes, especially early in the disease.

• Myopathies are disorders of muscle and can be inflammatory or congenital. Inflammatory myopathies usually involve proximal muscles and are associated with muscle pain or tenderness.

• Guillain–Barré syndrome (GBS) often starts with paresthesias in the fingers and toes, and nonspecific muscular pain, most often in the thighs. The pain is followed by ascending weakness, which results in trouble walking or balance problems.

• Infantile botulism is caused by colonization of the intestinal tract by spores of Clostridium botulinum. Many cases are linked to nearby construction projects, as soil harbors the spores. A prominent early manifestation is constipation.

The term “weakness” can refer to a general phenomenon that affects all or most of the body or may refer to a specific area, such as an extremity. The complaint can imply generalized fatigue, refusal to walk, increased clumsiness, loss of bowel or bladder function, or focal motor weakness. In infants, weakness can imply lethargy, poor feeding, or poor head control. Slowly progressive forms of weakness may be due to congenital disorders. The child may present at a time when the weakness is mild, yet progressive. Others may have paralysis at the time of presentation. The primary focus in this chapter is on weakness arising from neuromuscular disorders.

#### Pathophysiology

The pathophysiology of weakness varies with the etiology and the specific area affected. Terms that are applied to neuromuscular disorders include the following:

• Paresis implies a complete or partial weakness.

• Paraparesis is weakness of the lower half of the body.

• Quadraparesis is weakness involving all limbs.

• Hemiparesis is weakness of one side.1

• Quadriplegia is paralysis of all limbs; it usually results from a spinal cord lesion.

• Hemiplegia, involving one side of the body, generally results from a lesion in the brain.

Abnormalities of the neuromuscular system are further classified as arising from an upper or lower motor neuron unit. The upper motor neuron unit arises in the motor strip of cerebral cortex, traverses the corticospinal tract, and ends in the spinal cord adjacent to the anterior horn cell.2,3 Upper motor neuron diseases involving the cerebral cortex or spinal cord usually present with asymmetrical weakness that is contralateral to the lesion, and are associated with hyperreflexia, increased muscle tone, and the absence of atrophy or fasciculations.35 The lower motor neuron unit includes the anterior horn cells, peripheral nerve, neuromuscular junction, and muscle fibers. ...

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