TY  - JOUR
A1  - Cruccu, Giorgio
A1  - Pennisi, Elena M.
A1  - Antonini, Giovanni
A1  - Biasiotta, Antonella
A1  - Di Stefano, Giulia
A1  - La Cesa, Silvia
A1  - Leone, Caterina
A1  - Raffa, Salvatore
A1  - Sommer, Claudia
A1  - Truini, Andrea
T1  - Trigeminal isolated sensory neuropathy (TISN) and FOSMN syndrome: despite a dissimilar disease course do they share common pathophysiological mechanisms?
JF  - BMC Neurology
N2  - Background: Patients presenting with bilateral trigeminal hypoesthesia may go on to have trigeminal isolated sensory neuropathy, a benign, purely trigeminal neuropathy, or facial-onset sensory motor neuronopathy (FOSMN), a malignant life-threatening condition. No diagnostic criteria can yet differentiate the two conditions at their onset. Nor is it clear whether the two diseases are distinct entities or share common pathophysiological mechanisms. 
Methods: Seeking pathophysiological and diagnostic information to distinguish these two conditions at their onset, in this neurophysiological and morphometric study we neurophysiologically assessed function in myelinated and unmyelinated fibres and histologically examined supraorbital nerve biopsy specimens with optic and electron microscopy in 13 consecutive patients with recent onset trigeminal hypoesthesia and pain. 
Results: The disease course distinctly differed in the 13 patients. During a mean 10 year follow-up whereas in eight patients the disease remained relatively stable, in the other five it progressed to possibly life-threatening motor disturbances and extra-trigeminal spread. From two to six years elapsed between the first sensory symptoms and the onset of motor disorders. In patients with trigeminal isolated sensory neuropathy (TISN) and in those with FOSMN neurophysiological and histological examination documented a neuronopathy manifesting with trigeminal nerve damage selectively affecting myelinated fibres, but sparing the Ia-fibre-mediated proprioceptive reflex. 
Conclusions: Although no clinical diagnostic criteria can distinguish the two conditions at onset, neurophysiological and nerve-biopsy findings specify that in both disorders trigeminal nerve damage manifests as a dissociated neuronopathy affecting myelinated and sparing unmyelinated fibres, thus suggesting similar pathophysiological mechanisms.
KW  - amyotrophic-lateral-sclerosis
KW  - atrophy Kennedys-disease
KW  - trigeminal nerve
KW  - neuronopathy
KW  - trigeminal neuropathy
KW  - FOSMN
KW  - facial pain
KW  - Sjorgens-syndrome
KW  - reflex
KW  - afferents
KW  - neuralgia
KW  - pathways
KW  - humans
KW  - fibers
Y1  - 2014
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-114249
SN  - 1471-2377
VL  - 14
ER  -