Pandys autonomy

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The pandysautonomia is a rare immune-mediated, inflammatory disease of the autonomic nervous system . It develops acutely or subacutely within a few days to months and is then referred to as acute or subacute pandysautonomy . The terms autoimmune autonomic neuropathy , idiopathic autonomic neuropathy and panautonomic neuropathy are used synonymously .

The disease is considered to be a variant of Guillain-Barré syndrome (GBS), polyradiculitis with or without involvement of the autonomic nervous system. This is supported by microscopic examinations of vegetative ganglia with inflammatory changes such as in GBS. The fact that some patients are preceded by a viral infection suggests an immune-mediated genesis . A pathogen detection is rarely successful, however. In addition, the typically detectable increase in protein in the liquor (see also cytoalbuminal dissociation ) suggests an inflammatory genesis.

In some of those affected, autoantibodies against neuronal nicotinic acetylcholine receptors (α3β4) can be detected, the titer of which correlates with the severity of the symptoms, so that these antibodies are probably causing the disease. In this case, the clinical picture is analogous to (classic) myasthenia gravis , in which autoantibodies against the muscular nicotinic acetylcholine receptor are formed. The characteristic symptoms arise through the antibody-mediated blockade of the postsynaptic receptors.

Clinical picture

The extent to which the sympathetic and parasympathetic nervous systems are affected can vary. In one of the few studies of a larger group of patients, a total of 27 patients with pandys autonomy, 19 percent had a selective disorder of the parasympathetic nervous system. 81 percent had both sympathetic and parasympathetic failures. Clinically, this disease leads to tear , saliva and sweat secretion disorders , paralysis of the inner eye muscles , bladder and rectal disorders and orthostatic hypotension .

therapy and progress

Therapy is symptomatic , as no causal therapy is known. Due to the low number of cases, there are no controlled studies on the effectiveness of immunosuppressants , plasmapheresis and immunoglobulin therapies . Complete recovery from this condition is rare. The resolution is incomplete in 60–80% of cases and can take up to 2 years.

Individual evidence

  1. ↑ P. A: Low: Autonomic dysfunction in peripheral nerve disease . In: Muscle Nerve. 2003 Jun; 27 (6), pp. 646-661. PMID 12766975
  2. ↑ a b P. Berlit: Clinical Neurology. 2nd Edition. Springer-Verlag, 2006, ISBN 3-540-01982-0 , p. 421.
  3. ↑ a b M. Stöhr et al.: Neuromonitoring . Steinkopff-Verlag, 1999, ISBN 3-7985-1160-8 , p. 289.
  4. ^ Wilhelm Doerr et al.: Special pathological anatomy. Volume 13/8: Pathology of Peripheral Nerves. Springer-Verlag, 1999, ISBN 3-540-65612-X , p. 532.
  5. ↑ a b c d K. R. Müller-Vahl et al .: Idiopathic autonomic neuropathy; An important differential diagnosis disease . In: Dtsch Med Wochenschr. 1998 Nov 6; 123 (45), pp. 1343-1346, PMID 9835893
  6. ↑ Z. Wang, PA Low, J. Jordan, R. Freeman, CH Gibbons, C. Schroeder, P. Sandroni, S. Vernino: Autoimmune autonomic ganglionopathy: IgG effects on ganglionic acetylcholine receptor current. In: Neurology. Volume 68, number 22, May 2007, pp. 1917-1921, doi : 10.1212 / 01.wnl.0000263185.30294.61 , PMID 17536048 , PMC 2615674 (free full text).