Peripheral neuropathy and the role of nerve biopsy: A revisit.

BACKGROUND: Nerve biopsy has been widely used to investigate patients with peripheral neuropathy and in many centers, it is still a part of the diagnostic armamentarium. In this study, the histopathological spectrum of the nerve biopsies received is being revisited to analyze the various clinical and pathologic features and also to assess their relevance. MATERIALS AND METHODS: Retrospective analysis of the data retrieved was done for 74 cases of nerve biopsies. RESULTS: On the basis of the data and histopathological features, broad diagnoses were obtained in 52 cases and further categorized into biopsies being supportive for patient management (including acute and chronic axonopathies and demyelinating neuropathies) and biopsies considered essential for patient management (including vasculitic neuropathies, leprous neuropathies, hereditary neuropathies, and chronic inflammatory demyelinating neuropathies). Nine nerve biopsies did not show any abnormal histopathological features, while 13 nerve biopsies were found to be inadequate for diagnosis, both these groups were categorized as noncontributory. CONCLUSION: With advanced nerve conduction studies available, nerve biopsy is losing its relevance. However, in our experience, nerve biopsy did complement the clinical findings and nerve conduction studies, with which a close correlation is required to make the histopathology of nerve biopsy more relevant in terms of guiding further specific workup and management.

On the spectrum of leprosy neuropathies: multifocal inflammatory neuropathy heralding leprosy relapse.

We report a 52 year-old woman with a past history of lepromatous leprosy (14 years prior to our first evaluation) who presented with progressive weakness and severe arm/leg pain. CSF analysis revealed elevated protein level with normal cell count. Skin and sural nerve biopsy showed no bacilli. Immunomodulatory treatment led to major improvement on clinical, CSF and electrodiagnostic grounds, but after one year of treatment, skin test revealed leprosy relapse. To our knowledge, this is the first report of a multifocal inflammatory neuropathy heralding leprosy relapse. Extended neurological work-up may be important in unexplained neuropathy progression after leprosy treatment.

Arthritis as presenting manifestation of pure neuritic leprosy--a rheumatologist's dilemma.

OBJECTIVES: Leprosy classically presents with cutaneous and neurological manifestations. In diagnosed cases of leprosy, rheumatological involvement varies from 1% to 70%. A primary articular presentation without cutaneous manifestations is not yet known. Herein, we present our experience of five cases of leprosy that presented with predominant articular involvement in the absence of cutaneous manifestations. METHODS: The study was conducted in the Department of Clinical Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences located in the state of Uttar Pradesh, one of the nine endemic states in India. Case records of patients with a definite diagnosis of leprosy were screened for the presenting manifestations, pattern of articular involvement, tenosynovitis, neurological signs and symptoms. Reports of nerve conduction study (NCS), nerve and synovial biopsy and other diagnostic tests were retrieved from laboratory records. Available radiographs were examined for evidence of juxta-articular osteopenia and erosions. RESULTS: Case records of 11,740 patients were screened, of which 28 had a diagnosis of leprosy. Twenty patients had presented with rheumatological complaints primarily. Five of the patients who presented with inflammatory arthritis with/without tenosynovitis (n = 4) and tenosynovitis alone (n = 1) had pure neuritic leprosy. All of these patients had thickened peripheral nerves and abnormal NCS. Sural nerve biopsy confirmed the diagnosis of leprosy in all these cases. CONCLUSION: A combination of tenosynovitis and thickened nerves in association with symmetric polyarthritis should raise a suspicion of leprosy even in the absence of cutaneous features.

Leprosy: contribution of mast cells to epineurial collagenization.

BACKGROUND: Leprosy, a disease caused by Mycobacterium leprae, is an important health problem worldwide. It is responsible for an irreversible nerve damage in which fibrosis plays an important role. The existence of an interaction between mast cells and different fibrotic conditions has long been observed. Tryptase, the most abundant protein product of human mast cells, has been shown to be mitogenic for fibroblasts and to increase type I collagen production. PATIENTS AND METHODS: In order to explore the possible relationship between tryptase-rich mast cells and nerve fibrosis in leprosy, we studied 24 sural nerve biopsies of patients with leprous neuropathy. Mast cells stained with mouse antihuman mast cell antitryptase clone AA1 as well as fibrosis, were quantitatively estimated in both epi- and endoneurial compartments. RESULTS: There was a remarkable association between collagen increase and tryptase-rich mast cell density in the epineurium but not in the endoneurium of leprous nerves. CONCLUSION: Since the epineurium in leprosy is type I collagen rich, the present findings support a tryptase-rich mast cell contribution to epineurial collagenization in leprosy through their tryptase secretion.

Pseudoathetosis in a patient with leprosy.

A 35-year-old man with borderline tuberculoid leprosy developed Type I lepra reaction 12 days after anti-leprosy treatment. There was acute worsening of neuropathic symptoms and skin lesions. He developed severe sensory ataxia and pseudoathetosis resulting in marked disability. His symptoms significantly improved on corticosteroid therapy.

Clinicopathologic analysis of 124 biopsy-proven peripheral nerve diseases.

We reviewed dinical, histological and ultrastructural findings of 124 cases of sural nerve biopsy specimens to delineate the trends of peripheral nerve diseases in our institute. Eighty-one were men and 43 were women. We categorized them into five groups: specific diagnosis (66 cases, 53.2%), axonal degeneration type (47 cases, 37.9%), demyelinating type (4 cases, 3.2%), mixed axonal degeneration-demyelinating type (6 cases, 4.8%) and normal (1 case, 0.9%). Cases with specific diagnosis included 21 inflammatory demyelinating polyneuropathy (15 chronic inflammatory demyelinating polyradiculoneuropathy, 6 Guillain-Barre disease), 13 hereditary motor and sensory neuropathy (7 Charcot-Marie-Tooth type I, 6 Charcot-Marie-Tooth type II), 10 vasculitis, 6 toxic neuropathy, 4 leprosy, 3 diabetic neuropathy, 2 alcoholic neuropathy, 1 Fabry's disease and other specific diseases (5 cases). In our cases, the proportion of specific diagnoses was higher, while the proportion of demyelinating peripheral neuropathies and normal were lower than those of Western series. The results of this study indicate that 1) a dose clinicopathologic correlation is important to make a precise diagnosis of peripheral nerve biopsy, 2) Biopsy under strict indication may reduce unnecessary histologic examination, 3) There is no difference in disease pattern of peripheral neuropathy between Western people and Koreans.

[Suspected adult-onset Still's disease: unexpected manifestation of leprosy]. / Vermeintlicher Morbus Still des Erwachsenen: unerwartete Manifestation einer Lepra.

A 23-year old male from Sri Lanka was admitted to hospital with symmetrical inflammatory peripheral polyarthritis, fever of 39 degrees C and poly-lymphadenopathy. At first we suspected adult onset Still's disease. The histological findings from axillary lymph node biopsy strongly suggested the diagnosis of leprosy, for which we had had little evidence thus far. Typical skin lesions were absent, skin smears were negative and neurological symptoms only became obvious much later when fever and arthritis had subsided under anti-inflammatory treatment. At this time a right ulnar palsy developed, with atrophy of the interosseous muscles and thickening of the ulnar nerves at both medial epicondyles. Fite-stains of a sural nerve biopsy confirmed the diagnosis when mycobacteria were detected. Leprosy displays a clinico-pathological spectrum caused by variations in host resistance. A widely accepted classification is the five group system of Ridley and Jopling. At one extreme of this spectrum are patients with lepromatous or low resistance leprosy with numerous bacilli, and at the other those with high resistance or tuberculous leprosy where few or no bacilli are found. The numerous bacilli in the sural nerve biopsy classified the disease as lepromatous in our case. Of the various manifestations of the lepra reaction occurring in lepromatous leprosy, one is acute arthritis, but a more common one is erythema nodosum leprosum. Our patient's clinical presentation was interpreted to be a rheumatic manifestation of a type-2 reaction. This form of immunological response in leprosy is an immune complex syndrome and may mimic different rheumatic diseases.(ABSTRACT TRUNCATED AT 250 WORDS)

[Primary neural leprosy. Report of a case]. / Lepra neural primaria. A propósito de un caso.

We present the clinical manifestations and morphological characteristics in a case from a patient with familial history of leprosy that presented peripheral neuropathy without cutaneous lesions. The nerves affected showed a loss of myelinated and unmyelinated fibers as well as chronic inflammatory infiltrate. The presence of Hansen's bacillus was demonstrated in macrophages, Schwann cells, endothelial cells, and fibroblasts at light and ultrastructural levels. In this case, the demonstration of the bacillus in the biopsy of peripheral nerve confirmed the diagnosis.