-Leprosy is one of the most common diseases of the peripheral nerves. In some cases there is only neural involvement without skin changes (neuritic form). The neuropathy has often a distal stocking and glove distribution with thermal and pinprick anesthesia and preservation of proprioception. There is no weakness, the tendon reflexes may be preserved and sometimes the nerves are thickened. We reported 17 patients with a predominantly small-fiber polyneuropathy due to leprosy. All patients had distal temperature and pain anesthesia with different individual variations. The tendon reflexes were normal in seven patients and in eight there was thickening of the nerves. The nerve conduction was normal in three patients. Sural nerve biopsy consisted of: 1) inflammatory infiltrates, 2) vacuolated "foamy" cells, 3) fibrosis of endoneurium, perineurium, and epineurium, 4) partial or total loss of nerve fibers, 5) large number of bacilli. We concluded that in countries where leprosy is frequent, nerve biopsy is an obligatory procedure in patients with predominantly small-fiber polyneuropathy.KEY WORDS: leprosy, pure neuritic leprosy, small-fiber polyneuropathy, peripheral neuropathy, nerve biopsy. Polineuropatia de fibras-finas devido a lepra sem alterações cutâneas: estudo de 17 casosRESUMO -Em vários países tropicais a lepra constitui uma das principais causas de acometimento dos nervos periféricos. Em alguns casos somente os nervos são comprometidos, sem acometimento cutâneo (forma neurítica pura). Na polineuropatia há anestesia térmica e dolorosa com preservação da propriocepção. A força é normal, os reflexos profundos podem estar preservados e os nervos nem sempre estão espessados. Relatamos 17 pacientes com este tipo de polineuropatia. Todos tinham variados níveis de anestesia térmica e dolorosa distal nos membros. Os reflexos profundos estavam normais em sete pacientes e em oito havia leve espessamento dos nervos. A neurocondução foi normal em três pacientes. A biópsia do nervo sural revelou: 1) extenso infiltrado inflamatório, 2) células vacuoladas, 3) fibrose do endoneuro, perineuro e epineuro, 4) perda total ou parcial de fibras nervosas, 5) presença de bacilos da lepra. Concluímos que somente a biópsia de nervo permite o diagnóstico de pacientes com polineuropatia com acometimento predominante de pequenas fibras devida a lepra. PALAVRAS-CHAVE: lepra, lepra neurítica pura, polineuropatia de fibras finas, neuropatia periférica, biópsia de nervo.
RESUMO -A lepra constitui causa frequente de acometimento de nervos periféricos, em nosso meio. O sistema nervoso periférico é acometido por vezes sem que haja alterações cutâneas: é a chamada forma neurítica pura. Nessa variante, o nervo mais afetado é o ulnar. Nos casos de acometimento isolado de nervos periféricos somente a feitura de biópsia de nervo conduzirá ao diagnóstico. Assim, resolvemos realizar biópsia do ramo sensitivo superficial do nervo ulnar na mão em 17 pacientes com paresia ou paralisia desse nervo e espessamento do mesmo na altura do cotovelo. Os principais achados foram: redução do número de fibras mielínicas em 14 casos, infiltrado inflamatório em 13, fibrose em 12, desmielinização e remielinização em 9, presença de granuloma em 6 e visualização do Mycobacterium leprae em 5. Concluímos que a biópsia do ramo sensitivo superficial do nervo ulnar na mão é um bom meio diagnóstico de lepra em pacientes com acometimento desse nervo. PALAVRAS-CHAVE: lepra, nervo ulnar, biópsia.Ulnar nerve palsy in leprosy without skin changes: biopsy of the dorsal sensory branch in the hand ABSTRACT -Leprosy is one of the most common diseases of peripheral nerves in the world. In Brazil it is particularly frequent, being a major health problem. In tuberculoid leprosy the ulnar nerve is the most common affected nerve. Sometimes there are no skin changes. In these cases in spite of nerve thickening only the nerve biopsy is capable to make a specific diagnosis. We performed a biopsy in the dorsal sensory branch of the ulnar nerve in the hand in 17 patients with ulnar palsy with thickening of the nerve in the elbow, without skin changes. The pathological findings consisted mainly of: loss of fibers (14 cases), inflammatory infiltration (13), fibrosis (12), demyelination and remyelination (9), presence of granuloma (6) and presence of bacilli (5 cases). We conclude that in case of ulnar nerve palsy in leprosy without skin changes, the biopsy of the dorsal sensory branch of this nerve in the hand is a good procedure for the diagnosis of leprosy.KEY WORDS: leprosy, ulnar nerve, biopsy.A lepra é uma infecção crônica que atinge tecidos superficiais, especialmente a pele e o sistema nervoso periférico (SNP). É causada pelo Mycobacterium leprae, um bacilo álcool-ácido resistente. Em 1994 cerca de 2.4 milhões de pessoas estavam acometidas por esta moléstia, estando 80% concentradas em cinco países 1 , sendo que a Índia e o Brasil ocupavam os primeiros lugares. No Mercosul a prevalência da lepra é de 6,03/10000 habitantes 2 .
-Patients with leprosy may have only nerve involvement without skin changes. These cases are known as pure neural leprosy and can be seen in 10% of leprosy patients. Most patients have mononeuritic or multiple mononeuritic neuropathy patterns. The isolated lesion of the superficial peroneal nerve is uncommonly seen. We report a patient with involvement of this nerve in which there was no thickening of superficial nerves. The performed nerve biopsy showed inflammatory infiltration, loss of fibers and presence of Mycobacterium leprae. We believe that in prevalent leprosy countries we should take in mind the possibility of isolated pure neural leprosy in some patients without skin lesion. In these cases the diagnosis of leprosy is impossible on clinical grounds and nerve biopsy is mandatory.KEY WORDS: leprosy, hanseniasis, superficial peroneal nerve, nerve biopsy, pure neural leprosy.Acometimento isolado do nervo peroneiro superficial na lepra neurítica pura: relato de caso RESUMO -Em alguns casos de lepra podemos encontrar acometimento de nervos periféricos sem manifestações cutâneas. É a denominada lepra neurítica pura que pode ocorrer em até 10% dos casos desta doença. Na maioria das vezes predominam quadros de mononeuropatia ou mononeuropatia múltipla. O acometimento isolado do nervo peroneiro superficial tem sido pouco relatado. Referimos a uma paciente com acometimento isolado deste nervo sem hipertrofia de troncos nervosos. A biópsia realizada mostrou presença de infiltrado inflamatório, perda moderada de fibras e presença do Mycobacterium leprae. Acreditamos que, em países onde a lepra é endêmica, diante de acometimento isolado de nervos periféricos, deve-se pensar na possibilidade de se tratar da forma neurítica pura desta moléstia, mesmo em pacientes sem alterações dermatológicas. Nestes casos o diagnóstico só será possível com a realização de biópsia de nervo superficial. PALAVRAS-CHAVE: lepra, hanseníase, nervo peroneiro superficial, biópsia de nervo, lepra neurítica pura.Leprosy is one of the most common peripheral nerve diseases in the world. It usually affects the skin and the nerves. It is caused by the Mycobacterium leprae, an acid-fast bacillus.Although the prevalence of the disease is decreasing, leprosy continues to be a major cause of infective neuropathy in tropical and subtropical countries. Patients with leprosy may have only nerve involvement without obvious primary skin lesions. The so-called pure neuritic form is a well-recognized clinical entity, comprising 4 to 10% of patients with leprosy1 1,2 . In this pure neuritic form, the ulnar and the common peroneal nerves are the most frequently affected nerves 1,3 . Rare cases with superficial peroneal nerve involvement are referred in leprosy pure neuritic form 1 . We report a patient with superficial peroneal nerve compromise without nerve hypertrophy, and also without skin changes. CASEA 62 year-old woman was referred with progressive paresthesia in the outer aspect of the left leg. She had been well until three years early when sh...
Calf enlargement following sciatica is a rare condition. It is reported the case of a 28-year-old woman who complained of repeated episodes of lower back pain radiating into the left buttock and foot. One year after the beginning of her symptoms, she noticed enlargement of her left calf. X-ray studies disclosed L5-S1 disk degeneration. EMG showed muscle denervation with normal motor conduction velocity. Open biopsies of the gastrocnemius muscles were performed. The left gastrocnemius muscle showed hypertrophic type 2 fibers in comparison with the right gastrocnemius. Electron microscopy showed mildly increased number of mitochondria in these fibers. A satisfactory explanation for denervation hypertrophy has yet to be provided.
A case of Hodgkin's disease in which the first clinical manifestation was a radiculo-spinal compression is reported. The authors comment about the possible mechanisms to explain this radiculo-spinal compression in this granulomatosis.
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