Download PDF
Klinische Neurophysiologie 2017; 48(02): 73-83
DOI: 10.1055/s-0043-107751
Review
© Georg Thieme Verlag KG Stuttgart · New York

Sensible Neuronopathie – Fallberichte und Review

Sensory Neuronopathies – Case Reports and Review
Helmut Buchner
1   Klinikum Vest, Recklinghausen
› Author Affiliations
Further Information

Publication History

Publication Date:
02 June 2017 (online)

Also available at
    Permissions and Reprints

Zusammenfassung

Die sensible Neuronopathie ist klinisch gekennzeichnet durch Sensibilitätsstörungen dominant des Vibrations- und Lageempfindens, verursachend eine sensible Ataxie. Neurophysiologisch ist sie charakterisiert durch den Ausfall der sensiblen Nervenleitung bei normaler motorischer Neurografie. Neuropathologisch besteht eine Degeneration der Hinterstrangganglien. Eine „small fibre“ Neuropathie mit neuropathischen Schmerzen ist häufig. Neben hereditären und toxischen Ursachen sind Tumorerkrankungen (paraneoplastisch) und das Sjögren-Syndrom häufig, allerdings bleibt in rund der Hälfte aller Fälle die Ursache ungeklärt. Ist die Ursache behandelbar kann eine Verschlechterung verhindert werden und in wenigen Fällen kommt es zu Verbesserungen. An Fallberichten wird die sehr variable klinische Präsentation und der Verlauf der Erkrankung vorgestellt. Die Kriterien zur Differenzialdiagnose einer Polyneuropathie werden erklärt. Ein pragmatisches Vorgehen für die Suche nach der Ursache wird vorgeschlagen.

ABSTRACT

Sensory neuronopathy is clinically characterized by sensory disturbances, especially of the sensations of vibration and position, which causes ataxia. Neurophysiologically, it is characterized by the failure of sensitive nerve conduction while the neurography of motor nerves remains normal. Neuropathologically, there is a degeneration of the posterior ganglia. A "small fiber" neuropathy with neuropathic pain is common. In addition to hereditary and toxic causes, tumor diseases (paraneoplastic) and Sjögren syndrome are more frequent, but the cause remains unexplained in about half of all cases. If the cause is treatable, deterioration can be prevented, and in some cases there are improvements. The highly variable clinical picture and course of the disease is described by means of case reports. Criteria for the differential diagnosis of polyneuropathy are explained. A pragmatic approach to the search for the cause is proposed.

Schlüsselwörter

Neuronopathie - Ganglionopathie - Denny-Brown-Syndrom - Polyneuropathie - Neurografie - Sensible Störung - Ataxie - Pseudoathetose - Paraneoplasie - Sjögren-Syndrom

Key words

neuronopathy - ganglionopathy - denny brown syndrome - polyneuropathy - neurography - sensory disorder - ataxia - pseudoathetosis - paraneoplasia - sjögren’s syndrome
 

  • Literatur

  • 1 Denny-Brown D. Primary sensory neuropathy with muscular changes associated with carcinoma. J Neurol Neurosurg Psychiat 1948; 11: 73-87
    CrossrefPubMedGoogle Scholar
  • 2 Gwathmey KG. Sensory Neuonopathies. Muscle Nerve 2016; 53: 8-19
    CrossrefPubMedGoogle Scholar
  • 3 Camdessanché JP, Jousserand G, Ferraud K. et al. The pattern and diagnostic criteria of sensory neuronopathy: a case-control study. Brain 2009; 132: 1723-1733
    CrossrefPubMedGoogle Scholar
  • 4 Stöhr M, Pfister R. Klinische Elektromyographie und Neurographie – Lehrbuch und Atlas. 6. Aufl Kohlhammer; Stuttgart: 2014
    Google Scholar
  • 5 Grahmann F, Buchner H. Paraneoplastische sensible Polyneuropathie Denny-Brown mit nicht organischer. Tetraparese Klin Neurophysiol 2009; 40: 1-4
    PubMedGoogle Scholar
  • 6 Kimura J. Electrodiagnosis in diseases of nerve and muscle. 4. Ed. Oxford University Press; 2013
    Google Scholar
  • 7 Ross JS, Moore KR . Diagnostic imaging Spine Amirsys Salt Lake City 3. Ed. 2016;
    PubMed
  • 8 Szmulewicz DJ, McLean CA, Rodriguez ML. et al. Dorsal root ganglionopathy is responsible for the sensory impairment in CANVAS. Neurology 2014; 82: 1401-1415
    PubMedGoogle Scholar
  • 9 Antoine JC, Boutahar N, Lassablièr F. et al. Antifibroblast growth factor receptor 3 antibodies identify a subgroup of patients with sensory neuronopathy. J Neurol Neurosurg Psychiat 2015; 86: 1347-1355
    CrossrefPubMedGoogle Scholar
  • 10 Sillevis Smitt P, Grefkens J, de Leeuw B. et al Survival and outcome in 73 anti-Hu positive patients with paraneoplastic encephalomyelitis/sensory neuronopathy. J Neurol 2002; 249: 745-753
    CrossrefPubMedGoogle Scholar
  • 11 Pestronk A.http://neuromuscular.wustl.edu/index.html 2016;
    PubMed
  • 12 Pereira PR, Viala K, Maisonobe T. Sjögren sensory neuronopathy (Sjögren Ganglionopathy). Medicine 2016; 95: 1-6
    CrossrefPubMedGoogle Scholar
  • 13 Fox R. Sjögren’s syndrome. Lancet 2005; 366: 321-331
    CrossrefPubMedGoogle Scholar
  • 14 Mori K, Koike H, Misu K. et al. Spinal cord magnetic resonance imaging demonstrates sensory neuronal involvement nad clinical severity in neuronopathy associated with Sjögren’s syndrome. J Neurol Neurosurg Psychiat 2001; 71: 488-492
    CrossrefPubMedGoogle Scholar
  • 15 Yang J, Huan M, Jiang H. et al. Pure sensory Guillain-Barré syndrome: a case report and review oft he literature. Experimental and therapeutic medicine 2014; 8: 1397-1401
    CrossrefPubMedGoogle Scholar
  • 16 Denny-Brown D. Hereditary sensory radicular neuropathy. J Neurol Neurosurg Psychiat 1951; 14: 237-251
    CrossrefPubMedGoogle Scholar
  • 17 Knazan M, Bohlega S, Berry K. et al. Acute sensory neuronopathy with preserved SEPs and long-latency reflexes. Muscle nerve 1990; 13: 381-384
    CrossrefPubMedGoogle Scholar
  • 18 Verbiest HBC, van Woerkom TCAM, Dumas AM. et al. Subacute progressive sensory ataxic neuronopathy after Rickettsia conorii infection. Clin Neurol Neurosurg 1990; 92: 81-85
    CrossrefPubMedGoogle Scholar
  • 19 Nanetti L, Lauria G, Scaioli V. et al. Slowly progressive sensory hemisyndrome: unusual presentation of paraneoplastic sensory neuronopathy. J Peripher Nerv Syst 2010; 15: 73-74
    CrossrefPubMedGoogle Scholar