Contribution of nerve biopsy to unclassified neuropathy.

OBJECTIVES: To evaluate the diagnostic yield of nerve biopsy in patients with peripheral neuropathy of undetermined cause despite extensive diagnostic workup. METHODS: From November 2001 through January 2004, 38 patients underwent nerve biopsy because of unclassified neuropathy. RESULTS: The etiology of the neuropathies could be defined in 14 patients (37%), i.e. in 15% of chronic symmetric, 30% of chronic asymmetric, 50% of subacute symmetric and 62.5% of subacute asymmetric neuropathies. The biopsy was diagnostic in 6 patients (16%), where it showed a vasculitis, and supportive in 8 patients (21%). CONCLUSIONS: The contribution of nerve biopsy to the diagnosis of peripheral neuropathy was highest in acute and subacute asymmetric forms of neuropathy and lowest in chronic symmetric forms. The main indication for nerve biopsy remains the diagnosis of vasculitic neuropathy, a potentially treatable disorder.

Therapeutic strategies and management of desmoplastic infantile ganglioglioma: two case reports and literature overview.

INTRODUCTION: Desmoplastic infantile gangliogliomas (DIG) are rare cerebral glioneural tumors usually occurring in early childhood. DIGs are generally benign although rare cases with poor outcome are known. Total resection, if possible, is the treatment of choice, without further adjuvant therapy. After incomplete resection, adjuvant chemo-and/or radiotherapy is generally applied, despite the potential negative side effects in such young patients. CASE REPORTS: We describe two girls with DIG, one who twice underwent subtotal resection at 3 and 5 months, the other who underwent total resection at 2 years. Neither had adjuvant therapy and there was no tumor recurrence. CONCLUSIONS: Our own experience and a review of the literature suggest that in most DIGs adjuvant therapy is not justified even after incomplete resection. After tumor recurrence a second surgical intervention should be considered instead of adjuvant therapy. An exception may be made for rare, deep-seated DIGs, which are more aggressive and have a poorer outcome.