Peripheral nerve hyperexcitability

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Clinic

38 patients were included.

  1. Axonopathic-PNH (12 patients),
  2. Isolated nonparaneoplastic PNH (16 patients)
  3. Isolated paraneoplastic PNH (3 with thymoma and myasthenia gravis, 1 with thyroid carcinoma)
  • PNH clinical features were similar in groups 1 and 2.
  • We found an overall high prevalence of clinical autoimmunity (33% of group 1 and 63% of group 2) and systemic non-neuronal autoantibodies (42% of group 1 and 75% of group 2).
  • However, VGKC-Abs were only positive in 2 patients of group 2.
  • Ten patients underwent muscle biopsy, which showed inflammatory changes in 2 cases and nonspecific myopathic features in 8.


PNH is a heterogeneous disorder involving the peripheral nerves in patients with a high propensity for developing autoimmunity.

Associated muscle diseases are frequent in the form of myositis, myasthenia gravis, or nonspecific myopathic pathologic findings.

VGKC-Abs were uncommon in this series. [1]


Diagnostic features

Peripheral nerve hyperexcitability syndromes are a group of disorders characterized by

  • Muscle stiffness
  • Cramps
  • Muscle twitches
  • Involuntary abnormal electrical activity on needle EMG


DD

PNH syndromes are distinct from

  • Disorders of muscle stiffness originating in CNS, such as spasticity and stiff person syndrome
  • Muscle disorders such as the myotonias, rippling muscle disease
  • Glycogen storage diseases.


Despite the presence of several distinguishing clinical and electrophysiologic features in PNH syndromes, a significant overlap exists among the main three nerve hyperexcitability syndromes:

  1. Cramp-fasciculation syndrome
  2. Isaacs syndrome
  3. Morvan syndrome

To add to the confusion, the medical literature has used multiple terms to describe peripheral nerve hyperexcitability syndromes, including

  • Idiopathic generalized myokymia,
  • Acquired neuromyotonia,
  • Armadillo syndrome,
  • Syndrome of continuous muscle fiber activity, and
  • Quantal squander.

The electrophysiologic terms, including myokymia, neuromyotonia, continuous muscle fiber activity, continuous motor neuron discharges, and neurotonia, are used interchangeably and in an inconsistent fashion.

  1. Peripheral nerve hyperexcitability A clinical and immunologic study of 38 patients I. Rubio-Agusti, F. Perez-Miralles, T. Sevilla, N. Muelas, M.J. Chumillas, F. Mayordomo, I. Azorin, E. Carmona, F. Moscardo, J. Palau, L. Jacobson, A. Vincent, J.J. Vilchez, L. Bataller Neurology Jan 2011, 76 (2) 172-178; DOI: 10.1212/WNL.0b013e3182061b1e