Etiologic Spectrum of Biopsy-Proven Peripheral Neuropathies in Childhood from a Resource-Poor Setting
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There are only a few studies describing the etiologic spectrum of biopsy-proven peripheral neuropathies in children. This study reviewed the clinical, electrophysiological, and pathologic profile of 239 children (≤18 years of age) who have undergone nerve biopsy in a tertiary care centre for neurologic disorders and analyzed the etiologic spectrum and utility of nerve biopsy. The clinical profile, neuropathologic findings, and other investigations were combined to infer the final diagnosis. Neuropathy was detected in 199 biopsies; axonal pathology in 43%; demyelination in 41%; mixed pattern in 8%; and nonspecific findings in 8%. The major diagnostic categories included hereditary neuropathies (48%), heredodegenerative and metabolic disorders (27%), and inflammatory neuropathies (12%). Nerve biopsy proved most helpful in diagnosis of demyelinating and inflammatory neuropathies, reiterating its usefulness in specific situations.Keywords:
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Diabetic peripheral neuropathy is one of the most frequent complications of diabetes.There are many methods for the assessment of diabetic peripheral neuropathy including clinical scoring systems,such as Neuropathy Disability Score and Michigan Neuropathy Screening Instrument,can be used to assess and quantify severity of neuropathy;screening methods,such as tuning fork and Semmes-Weinstein monofilament;diagnostic methods,such as nerve conduction studies and quantitative sensory testing,can be used to document and monitor neuropathy in the clinic;some morphological methods like nerve biopsy and skin biopsy,are mainly used in laboratory research,their clinical application needs further study.
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Paclitaxel (PTX) is frequently used for a chemotherapy of breast cancer and gynecologic cancer. Besides a bone-marrow depression and hypersensitive reaction, the peripheral neuropathy is one of the serious adverse events of PTX. The mechanism of peripheral neuropathy has not been clarified, and few agents have been reported to be effective for the treatment and the prevention of that. Recently, it has been reported that Gosya-jinki-gan is useful for the PTX induced peripheral neuropathy, so we carried a retrospective study(n=82)to evaluate the effectiveness of Gosya-jinkigan with the medical records. It is suggested that peripheral neuropathy developed more rapidly in sequential administration of PTX every week than in administration in 4 weeks cycles consisting of 3 weeks on and 1 week off(5.4w vs. 9.4w). We have also found that Gosya-jinki-gan was possibly effective for the treatment and the prevention of peripheral neuropathy. Additionally Gosya-jinki-gan might be more effective for peripheral neuropathy when it is administered from the beginning of chemotherapy including PTX.
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ABSTRACT Introduction : The utility of repeat muscle biopsy has not been adequately evaluated. Methods : A retrospective review was undertaken of 144 repeat muscle biopsies performed from 1980 to 2017. Repeat biopsy was considered clinically relevant if it provided a new diagnosis, changed the existing diagnosis, or led to treatment changes or further investigations. Results : Repeat biopsy was abnormal in 118 cases, different from the initial biopsy in 67 cases, and specific in 40 cases. Factors with a significant effect on clinical relevance of the repeat biopsy ( P < 0.05) were an abnormal, specific, or inflammatory initial biopsy, proximal muscle weakness, absence of myalgia, and a repeat biopsy that is different, specific, or consistent with polymyositis or inclusion body myositis. Conclusions : Utility of repeat biopsy was limited to weak patients whose initial biopsy showed inflammatory myositis. Ongoing advances in the diagnosis of immune inflammatory myopathies have led to evolution of the role of repeat biopsy. Muscle Nerve , 2019
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This chapter is a basic introduction to the techniques used for processing and analyzing peripheral nerve biopsies. The most informative method depends on the clinical context of the biopsy. Procedures for each individual technique are described, with a discussion of the fixation and handling required for each. Knowledge of the clinical differential diagnosis aids in the decision on which methods are likely to be the most informative; all methods may not be utilized in individual biopsies. Selected references are provided for detailed descriptions.
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