Leila Chimelli

Value of nerve biopsy in the diagnosis and follow-up of leprosy: the role of vascular lesions and usefulness of nerve studies in the detection of persistent bacilli.

Abstract Nerve biopsy specimens from 53 patients with leprosy and neuropathy were taken from the sural, the dorsal branch of the ulnar, or the superficial radial nerves and processed for light and electron microscopy. There was inflammation in 40 cases (75%), 7 with a granulomatous reaction, various stages of fibrosis in 35 (66%), and endoneurial vascular neoformation in 7. In two cases, small focal infarcts were associated with marked endoneurial inflammation compressing the vessels, in addition to endoneurial lymphocytic vasculitis. Most had an axonal neuropathy of varying degree, some with total fibre loss, others with predominant small myelinated and unmyelinated fibre loss. Signs of demyelination and remyelination were the main findings in 9 cases (17%). Bacilli were present in endothelial, perineurial, Schwann cells and in macrophages. On two occasions, they lost their alcohol acid resistance, were suspected in semithin sections, and confirmed ultrastructurally. The biopsy was decisive for the diagnosis of leprosy in 15 cases (28%), most without skin lesions. We evaluated the effectiveness of the treatment in 20 (37.7%), 12 without and 8 with bacilli, despite negativity in the skin. The diagnosis of leprosy based on skin lesions was confirmed with the nerve biopsy in 9 cases, 6 had an inflammatory neuropathy suggestive of leprosy in the absence of bacilli, and 3 had nonspecific changes in the sural nerve since the neuropathy was in the upper limbs. We conclude that nerve biopsy is indicated for the diagnosis of leprosy in cases without clinically visible skin lesions and to evaluate the effectiveness of the treatment. In these cases the ultrastructural studies are important for recognition of the bacilli. Vascular lesions may play an important role in the progression of the nerve damage, including the occurrence of focal nerve infarcts which, to our knowledge, have not been previously reported in association with leprosy.

Tapia's syndrome caused by Paracoccidioidis brasiliensis

Tapias syndrome is due to extracranial involvement of the XIIth nerve and the recurrent laryngeal branch of the Xth nerve. There is ipsilateral paralysis of vocal cords, soft palate and tongue. The main causes are parotid and other tumors or injuries to the high neck. We describe here a Brazilian female patient who presented with a lesion in the nasal mucosa, and soon after had dysphagia and dysphonia. Examination revealed paralysis of the soft palate, vocal cords and tongue ipsilaterally. Microscopic examination of the lesion in the nasal mucosa revealed the presence of the fungus. The patient was treated with sulfonamide and ketoconasol.

Polineuropatia hipoglicêmica: registro de caso com insulinoma

A case of a young man who presented symptoms and clinical signs of polyneuropathy that occurred in connection with recurrent hypoglycemic episodes is reported. The hypoglycemia was probably caused by a pancreatic islet tumor. There were symmetric weakness and wasting of hands and feet, absent tendon reflexes and glove and stocking loss of sensation. Electromyographic studies showed denervation potentials with slight reduction of nerve conduction velocities. Sural nerve biopsy studied by optic and electronic microscopy showed axonal degeneration without signs of demyelination or remyelination. There are only 30 similar cases reported in the literature. According to experimental findings, the authors believe that glucopenia is the mechanism responsible for the development of the neuropathy, and that at present time there is no evidence for a direct insulin effect.A case of a young man who presented symptoms and clinical signs of polineuropathy that occurred in connection with recurrent hypoglycemic episodes is reported. The hypoglycemia was probably caused by a pancreatic islet tumor. There were, symmetric weakness and wasting of hands and feet, absent tendon reflexes and glove and stocking loss of sensation. Electromyographic studies showed denervation potentials with slight reduction of nerve conduction velocities. Sural nerve biopsy studied by optic and electronic microscopy showed axonal degeneration without signs of demyelination or remyellnation. There are only 30 similar cases reported in the literature. According to experimental findings, the authors believe that glucopenia is the mechanism responsible for the development of the neuropathy, and that at present time there is no evidence for a direct insulin effect.

Peripheral neuropathy in hypereosinophilic syndrome with vasculitis

A 53-year-old woman with non-productive cough of unexplained aetiology for two years, developed a sub-acute symmetrical polyneuropathy involving all four limbs, accompanied by fever, cutaneous rash and myalgia in lower limbs. Laboratory studies revealed a leukocytosis with 70% eosinophils and excluded any cause for the hypereosinophilia. An echocardiogram showed increase in thickness of the atrial septum. Motor and sensory conduction velocity were reduced in ulnar and median nerve and unrecordable in peroneal and tibial nerves. A sural nerve biopsy showed an axonal degeneration involving myelinated and unmyelinated fibers as well as a vasculitis with fibrinoid necrosis and perivascular infiltration of eosinophils. There was considerable clinical and laboratory improvement with the use of steroids. The differential diagnosis between idiopathic hypereosinophilic syndrome and other disorders known to course with vasculitis and hypereosinophilia is discussed.

Polineuropatia por triclorfon registro de um caso com estudo eletrofisiodógico e histopatológico do nervo sural

The authors observed a patient who worked in a farm and suffered an organophosphate intoxication (trichlorphon). The immediate effect was manifested by vomiting and abdominal cramps. Three months later he presented a distal symmetric sensorimotor (predominantly motor) neuropathy with distal muscle atrophy. Electromyography has revealed denervation changes in every muscle studied and the sensory and motor nerve conduction was slowed in arms and legs. The sural nerve biopsy specimen studied by light microscopy with semi-thin section and teased fiber preparation showed axonal degeneration. The ultrastructural studies of the axonal alterations consisted of degeneration of the neurofilaments and the neurotubules with granular appearance of the axoplasm.The authors observed a patient who worked in a farm and suffered an organophosphate intoxication (trichlorfon). The immediate effect was manifested by vomiting and abdominal cramps. Three months later he presented a distal symmetric sensorimotor (predominantly motor) neuropathy with distal muscle atrophy. Electromyography has revealed denervation changes in every muscle studied and the sensory and motor nerve conduction was slowed in arms and legs. The sural nerve biopsy specimen studied by light microscopy with semi-thin section and teased fiber preparation showed axonal degeneration. The ultrastructural studies of the axonal alterations consisted of degeneration of the neurofilaments and the neurotubules with granular appearance of the axoplasm.

Polineuropatia devida ao óxido de etileno registro de caso com estudo clínico, eletrofisiológico e histopatológico

A man who worked as an operator in a factory of sterilization of heat-sen-sitive materials has been exposed to ethylene oxide for seven years. He developed a mild sensori-motor polyneuropathy. The electromyography and nerve conduction studies showed an axonal degenerative type of neuropathy. The sural nerve biopsy revealed mild loss of myelinated fibers, some fibers with axonial degeneration, some clusters of regeneration and few rows of myelin ovoids in the teased nerve fiber preparation. This is the first report of ethylene-oxide polyneuropathy in Brazil.A man who worked as an operator in a factory of sterilization of heat-sensitive materials has been exposed to ethylene oxide for seven years. He developed a mild sensori-motor polyneuropathy. The electromyography and nerve condition studies showed an axonal degenerative type of neuropathy. The sural nerve biopsy revealed mild loss of myelinated fibers, some fibers with axonal degeneration, some clusters of regeneration and few rows of myelin ovoids in the teased nerve fiber preparation. This is the first report of ethylene-oxide polyneuropathy in Brazil.

Chronic recurrent Guillain-Barré syndrome: report of 3 cases

The classical Guillain-Barré syndrome is an acute or subacute polyradiculo-neuropathy whose main clinical features are progressive weakness of the limbs, decrease or absence of tendon reflexes, and sensory changes. Although in most of the cases there is complete recovery in weeks or months, some patients have a slow and progressive relapsing course and present thickening of the peripheral nerves. In this paper we describe three cases of the chronic and relapsing variety of Guillain-Barré syndrome, two of which had prominent hypertrophic changes in the peripheral nerves with onion bulb formations. The clinical and pathological features of this disease are reviewed. The three patients improved with the use of steroids.

Correlação clínico-patológica em 78 biópsias de nervo sural

Peripheral nerve biopsies when processed with conventional techniques for paraffin embedding usually do not provide sufficient data for the diagnostic conclusion. However, if the nerve is processed fcr resin embedding for semi and ultra-thin sections and teasing of fibres, several aspects can be analysed including quantitative and morphometric data. We studied the sural nerve biopsy of 78 patients examined at the Antonio Pedro University Hospital, Niteroi RJ, applying those techniques and we found that in 55 cases (70.5%) the pathologic diagnosis was conclusive, in 11 (14.1%) although the nerve had abnormalities it was not possible to establish a diagnosis, and in 12 (15.4%) the nerve was normal. In 68 cases there was a clinical diagnosis which was confirmed in 49 but not in the remaining 19, since 8 had non-specific changes and 11 were normal. From the 10 cases which did not have a clinical diagnosis the biopsy was conclusive in 6, showed non-specific changes in 4, and was normal in 1 case. The pathologic conclusion in most of our cases was possible because not only we had the clinical data but all the nerves were processed for resin embedding.Peripheral nerve biopsies when processed with conventional techniques for paraffin embedding usually do not provide sufficient data for the diagnostic conclusion. However, if the nerve is processed for resin embedding for semi and ultra-thin sections and teasing of fibres, several aspects can be analysed including quantitative and morphometric data. We studied the sural nerve biopsy of 78 patients examined at the Antonio Pedro University Hospital, Niterói RJ, applying those techniques and we found that in 55 cases (70.5%) the pathologic diagnosis was conclusive, in 11 (14.1%) although the nerve had abnormalities it was not possible to establish a diagnosis, and in 12 (15.4%) the nerve was normal. In 68 cases there was a clinical diagnosis which was confirmed in 49 but not in the remaining 19, since 8 had non-specific changes and 11 were normal. From the 10 cases which did not have a clinical diagnosis the biopsy was conclusive in 6, showed non-specific changes in 4, and was normal in 1 case. The pathologic conclusion in most of our cases was possible because not only we had the clinical data but all the nerves were processed for resin embedding.