Recent histopathological studies in leprosy, with particular reference to early diagnosis and leprous neuropathy.

In histopathological studies in leprosy, two important areas were identified in recently published work. They are early diagnosis and neuropathy. In histopathological examination, finding of M. leprae in tissues and/or granulomatous destruction of nerves are the two important findings to confirm the diagnosis. Immunopathological staining of M. leprae, PCR amplification of M. leprae antigen and S100 staining of Schwaann cells have considerably enhanced the sensitivity of histopathological diagnosis. If the two clinical findings such as hypopigmented patches with impaired sensation and thickened nerves accompanied by loss of sensation are the only ones that are taken into account for diagnosis, then a significant number of early patients will be missed. It is pointed out that biopsy examination of skin and nerves, when necessary, and skin-smear studies are indispensable diagnostic procedures. In the study of leprous neuropathy, there are several studies trying to decipher the entry of M. leprae into Schwann cells. The sharing of antigens between M. leprae and surface membrane of Schwann cells may be an important factor. However, there is much more to be learned in this area. In the control and prevention of neuritis, although corticosteroids administered along with multi-drug therapy was helpful, the benefit was not sustained.

An immunohistochemical, clinical and electroneuromyographic correlative study of the neural markers in the neuritic form of leprosy

The nerve biopsies of 11 patients with pure neuritic leprosy were submitted to routine diagnostic procedures and immunoperoxidase staining with antibodies against axonal (neurofilament, nerve growth factor receptor (NGFr), and protein gene product (PGP) 9.5) and Schwann cell (myelin basic protein, S-100 protein, and NGFr) markers. Two pairs of non-adjacent histological cross-sections of the peripheral nerve were removed for quantification. All the fascicles of the nerve were examined with a 10X-ocular and 40X-objective lens. The immunohistochemistry results were compared to the results of semithin section analysis and clinical and electroneuromyographic data. Neurofilament staining was reduced in 100 percent of the neuritic biopsies. NGFr positivity was also reduced in 81.8 percent, PGP staining in 100 percent of the affected nerves, S100 positivity in 90.9 percent, and myelin basic protein immunoreactivity in 90.9 percent. Hypoesthesia was associated with decreased NGFr (81.8 percent) and PGP staining (90.9 percent). Reduced potential amplitudes (electroneuromyographic data) were found to be associated with reduced PGP 9.5 (63.6 percent) and nerve fiber neurofilament staining (45.4 percent) by immunohistochemistry and with loss of myelinated fibers (100 percent) by semithin section analysis. On the other hand, the small fibers (immunoreactive dots) seen amid inflammatory cells continued to be present even after 40 percent of the larger myelinated fibers had disappeared. The present study shows an in-depth view of the destructive effects of leprosy upon the expression of neural markers and the integrity of nerve fiber. The association of these structural changes with the clinical and electroneuromyographic manifestations of leprosy peripheral neuropathy was also discussed.

Pure neural leprosy presenting with multiple nerve abscesses.

A 22-year-old male student presented to us with patchy sensory loss over the dorsum of right foot of 6 months' duration and swelling over the left index finger of 2 months' duration. Subsequently, a week later, he developed a painful swelling over the left forehead and right leg. On examination, tender soft, fluctuant subcutaneous saccular swellings were seen varying in size from 2x2 cm to 5x5 cm over the left supratrochlear, left radial cutaneous nerve, left digital nerve, right superficial peroneal nerve and left saphenous nerve. The nerves were tender and thickened above and below these cold swellings (cold nerve abscesses). Nerve biopsy of the left radial cutaneous nerve showed granulomatous infiltrate of epithelioid cells, lymphocytes and caseation necrosis of nerve. No bacilli were demonstrated with acid-fast stain. On the basis of the above findings, a diagnosis of pure neuritic leprosy (BT spectrum) in type 1 lepra reaction with multiple nerve abscesses was made. We present this case of pure neuritic leprosy exhibiting multiple nerve abscesses, for its rarity.

Polineuropatia do paciente crítico: registro de caso / Critical illness polyneuropathy: case report

A polineuropia do paciente crítico é de natureza axonal, aguda e de predomínio motor, desenvolvendo-se em pacientes sépticos ou com falência de múltiplos órgãos que geralmente necessitam de ventilação mecânica em unidade de tratamento intensivo. Seu diagnóstico é corroborado por achados eletroneuromiográficos e, geralmente, a evolução é satisfatória e rápida. Relatamos o caso de uma paciente de 35 anos que, após internação em unidade de tratamento intensivo devido a septicemia e retirada de feto morto, desenvolveu tetraparesia com melhora importante em quatro meses. A eletroneuromiografia mostrou diminuição de amplitudes de potenciais sensitivos e motores, ondas positivas e fibrilações. A biópsia de nervo sural evidenciou neuropatia axono-mielínica em atividade. Estando esses achados condizentes com os da literatura, acreditamos tratar-se de caso de polineuropatia do paciente crítico.

Membranous glomerulonephritis and the nephrotic syndrome in a patient with Landry-Guillain-Barré-Strohl syndrome

Membranous glomerulonephritis and the nephrotic syndrome concurrent with the Miller-Fisher variant of the Landry-Guillain-Barré-Strohl syndrome (LGBS), acute post-infective polyneuritis, is reported in a 49-year-old man. The onset of heavy proteinuria coincided with the development of the neurological disturbance. While immunosuppressive therapy appeared to hasten improvemente in the neurological disease, no such improvemente occurred in the glomerulopathy

Neurites: parte IV / Neurites: part IV

Esta parte do curso visa der um apanhado geral e objetivo sobre o problema das neurites, sejam elas poli ou mononeurites. Após uma introduçäo e uma resenha anatômica säo estudadas a patologia geral das neurites e algumas doenças sistêmicas que produzem neurites múltiplas

Polimiositis subaguda: observaciones clinicopatologicas / Subacute polymyositis clinicopathological observations

Se estudian tres casos de poliomiositis, aguda subaguda tipo II, dos de ellos fatales. Las edades fluctuaron entre 14 y 58 anos. Ademas del analisis de los detalles clinicos y paraclinicos se ha hecho un minucioso estudio histopatologico de diversos grupos musculares y se subraya el interes que podrian tener las alteraciones de la musculatura lisa del esofago y del colon. Hemos revisado ampliamente y consignado algunos de los conceptos actuales sobre la etiopatogenia de la polimiositis