Axonal and perikaryal involvement in chronic inflammatory demyelinating polyneuropathy
- M Nagamatsua,
- S Teraob,
- K Misua,
- M Lia,
- N Hattoria,
- M Ichimuraa,
- M Sakaic,
- H Yamamotoc,
- H Watanabed,
- S Rikue,
- E Ikedaf,
- J Hataf,
- M Odag,
- M Satakeh,
- N Nakamurai,
- S Matsuyaj,
- Y Hashizumek,
- G Sobuea
- aDepartment of Neurology, Nagoya University School of Medicine, Nagoya, Japan, bFourth Department of Internal Medicine, Aichi Medical University, Aichi, Japan, cDepartment of Neurology, Fujita Health University School of Medicine, Toyoake, Japan, dDepartment of Neurology, Nogoya Daini Red Cross Hospital, Nagoya, Japan, eDepartment of Neurology, Chukyo Hospital, Nagoya, Japan, fDepartment of Pathology, Keio University School of Medicine, Tokyo, Japan, gDepartment of Pathology, Tokyo Metropolitan Neurological Hospital, Tokyo, Japan, hDepartment of Neurology, Faculty of Medicine, Kyushu University, Fukuoka, Japan, iDepartment of Pathology, Yokohama Municipal Citizens Hospital, Yokohama, Japan, jDepartment of Diagnostic Pathology, NTT Kanto Teishin Hospital, Tokyo, Japan, kInstitute for Medical Science of Aging, Aichi Medical University, Aichi, Japan
- Dr G Sobue, Department of Neurology, Nagoya University School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya 466–8550, Japan. Telephone 0081 52 741 2111; fax 0081 52 744 2384.
- Received 9 September 1998
- Revised 16 December 1998
- Accepted 22 December 1998
Abstract
OBJECTIVES To assess the extent of loss of myelinated nerve fibres and spinal motor neuron loss in chronic inflammatory demyelinating polyneuropathy (CIDP), a clinicopathological study was conducted on biopsied sural nerves and necropsied spinal cords from patients with CIDP.
METHODS The myelinated fibre pathology of 71 biopsied sural nerves and motor neuron pathology of nine necropsied spinal cords at L4 levels in patients with CIDP were quantitatively and immunohistochemically assessed.
RESULTS Myelinated nerve fibre density was significantly diminished to 65.4% of the control values (p <0.0001), correlating inversely with the extent of segmental demyelination and remyelination (r = −0.43, p < 0.0005) and duration of illness (r = −0.31, p < 0.01). Numbers of large spinal motor neurons in CIDP were variably but significantly diminished (range from 46.0 to 97.6% of the age matched control value (p < 0.005)), and reactive astrogliosis was evident in the ventral horn in CIDP. The frequency of ventral horn neurons exhibiting central chromatolysis and the accumulation of phosphorylated high molecular weight neurofilament protein was significantly higher in CIDP than in controls (p<0.01 and p<0.05).
CONCLUSIONS The loss of nerve axons and spinal motor neurons is common in CIDP, and extensive in some cases. These neuronal and axonal losses may influence the functional prognosis in CIDP.
