Trigeminal isolated sensory neuropathy (TISN) and FOSMN syndrome: despite a dissimilar disease course do they share common pathophysiological mechanisms?

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• 作者：Giorgio Cruccu (1)
  Elena M Pennisi (2)
  Giovanni Antonini (3)
  Antonella Biasiotta (1)
  Giulia di Stefano (1)
  Silvia La Cesa (1)
  Caterina Leone (1)
  Salvatore Raffa (4)
  Claudia Sommer (5)
  Andrea Truini (1)
  
  1. Department of Neurology and Psychiatry ; Sapienza University ; Rome ; Italy
  2. Neurology Division ; Neurosciences Department ; San Filippo Neri Hospital ; Rome ; Italy
  3. Department NESMOS ; Sant鈥橝ndrea Hospital ; Rome ; Italy
  4. Cellular Diagnostics Unit ; Department of Clinical and Molecular Medicine ; Sant鈥橝ndrea Hospital ; Rome ; Italy
  5. Department of Neurology ; University of W眉rzburg ; W眉rzburg ; Germany
  
• 关键词：Trigeminal nerve ; Neuronopathy ; Trigeminal neuropathy ; FOSMN ; Facial pain
• 刊名：BMC Neurology
• 出版年：2014
• 出版时间：December 2014
• 年：2014
• 卷：14
• 期：1
• 全文大小：1,698 KB
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• 刊物主题：Neurology; Neurochemistry; Neurosurgery;
• 出版者：BioMed Central
• ISSN：1471-2377

文摘

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.