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A Clinical Study on the Utility of Nerve Biopsy in Peripheral Neuropathy

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A Clinical Study on the Utility of Nerve Biopsy in Peripheral Neuropathy A CLINICAL STUDY ON THE UTILITY OF NERVE BIOPSY IN PERIPHERAL NEUROPATHY Thesis submitted for the partial fulfilment for the requirement of the degree of DM Neurology DR. JITESH GOEL DM NEUROLOGY RESIDENT 2014–2016 DEPARTMENT OF NEUROLOGY SREE CHITRA TIRUNAL INSTITUTE FOR MEDICAL SCIENCES AND TECHNOLOGY, TRIVANDRUM, KERALA 695011 i DECLARATION I, Dr Jitesh hereby declare that the thesis “A CLINICAL STUDY ON THE UTILITY OF NERVE BIOPSY IN PERIPHERAL NEUROPATHY” was undertaken by me under the guidance and supervision of Dr MD Nair, Senior Professor and Head of Department, Department of Neurology at the Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram. Dr.Jitesh Goel Thiruvananthapuram Senior Resident Date: Dept. of Neurology SCTIMST Thiruvananthapuram ii CERTIFICATE This is to certify that the thesis titled “A CLINICAL STUDY ON THE UTILITY OF NERVE BIOPSY IN PERIPHERAL NEUROPATHY”, is the bonafide work of Dr Jitesh Goel, Senior Resident, DM Neurology and has been done under my direct guidance and supervision at the Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram. He has shown keen interest in the research project and actively participated in all its phases. Thiruvananthapuram Dr MD Nair (Guide) Date: Senior Professor and Head of Department Department of Neurology, SCTIMST. Thiruvananthapuram iii CONTENTS Sl. No. Title Page No. 1 Introduction 1 2 Review of Literature 3 3 Aim of The Study 32 4 Materials And Methods 32 5 Results 34 6 Discussion 62 7 Conclusion 72 8 References 75 9 Annexures 84 IEC Approval Proforma iv INTRODUCTION Peripheral neuropathy is among the common disorders in patients attending neuromuscular clinic. Systematic approach comprising a comprehensive clinical history, thorough neurological and systemic examination, nerve conduction studies, EMG and relevant biochemical tests should be undertaken in all cases. Nerve biopsy is indicated with a strong suspicion of disorders, like amyloidosis, vasculitis, leprosy, and tumor infiltration. Nerve biopsy is useful for atypical presentations of CIDP, and is helpful in exclusion of other etiologies. The yield of nerve biopsy result is dependent on number of factors, including appropriate selection of patients for biopsy, expertise of the laboratory, and techniques used in the analysis. A prospective study by Gabriel etal has shown altered management in nearly 60 % of cases after nerve biopsy, and nerve biopsy was more diagnostic in severe demyelinating, distal asymmetric, and multifocal type of neuropathy 1, similar results have been shown in another retrospective study 2. The yield of nerve biopsy in vasculitic neuropathy is around 20 %, as observed in some other studies 3,4. Combined nerve and muscle biopsy has shown improved yield in vasculitic neuropathy, due to the more frequent involvement of the peroneal nerve and the involvement of muscular arteries in vasculitis neuropathy. Higher yield in vasculitis neuropathy has 1 been shown to be useful by performing a biopsy of the superficial peroneal nerve combined with a peroneus brevis muscle biopsy, confirmed in a multicenter prospective study5. Nerve biopsy is more contributive in the diagnosis in multifocal neuropathy than in the other patterns of neuropathy. 2 REVIEW OF LITERATURE In a community-based study conducted in Bangalore, the age adjusted prevalence rate of neuropathy in population was found to be 0.067 % 6, and in another study conducted in Parsi community in Bombay, prevalence rate was found to 2.3 % 7. Prevalence of peripheral neuropathy in Community based surveys in Italy have been reported between 1-3% 8. In the Italian field screen study for distal symmetrical polyneuropathy, out of 4191 subjects aged more than 55 years, 888 patients had peripheral neuropathy and of these nearly 47.3 % of patients had diabetes mellitus. Detailed analysis revealed a prevalence of distal symmetric polyneuropathy to be 3.3 – 3.6 per 100 population. Most common symptoms reported by these patients were distal paraesthesias and muscle cramps. Most common signs reported were impaired reflexes and impaired sensations9. The approach to patients with suspected polyneuropathy starts with a comprehensive clinical history and identification of risk factors. Detailed neurologic examination and electro-diagnostic studies are used to identify the distribution of the neuropathy and to characterize the process as sensory (large or small fiber types, or mixed), motor, sensorimotor, or autonomic, and as axonopathic, demyelinating, or combined. The decision for nerve biopsy is taken on the basis of these results. Nerve biopsy remains a useful diagnostic tool in cases of multifocal, asymmetrical, painful or autonomic neuropathies where vasculitis, sarcoidosis and amyloidosis are diagnostic possibilities. 3 Besides, nerve biopsy is also useful to detect or confirm histologically other causes of peripheral neuropathy such as infections (e.g. leprosy, cytomegalovirus infection), tumors like lymphoma or neurinoma, and granulomatous diseases. In a prospective study by C M Gabriel etal, diagnostic utility of sural nerve biopsy in 50 consecutive patients with peripheral neuropathy was studied. Nerve biopsy was useful in nearly 60 % cases, especially in cases with demyelinating neuropathy and multiple mononeuropathy, besides helpful in confirmation of clinical diagnosis in nearly 70 % cases. Nerve biopsy revealed an otherwise unsuspected diagnosis in 14% of the patients and in 16% the biopsy findings were non - contributory. This study also showed that the yield of nerve biopsy done after site selection as guided by clinical and electrodiagnostic findings are higher 1. In another prospective study of 38 patients who underwent nerve biopsy, nerve biopsy proved to be useful in defining the etiology in 14 patients (37%). The diagnostic yield of nerve biopsy was highest in acute/ sub acute symmetric and sub acute asymmetric neuropathies, followed by chronic symmetric and chronic asymmetric neuropathies. The biopsy was diagnostic in 6 patients (16%), in cases where histopathological features were suggestive of vasculitis, and was supportive of diagnosis in 8 patients (21%) 10. In the current scenario, due to availability of newer genetic, and pathological diagnostic modalities, and recognition of newer diagnostic entities, the percentage of cases of neuropathy of undetermined etiology has considerably decreased. However, 4 inspite of rapid advances, etiology of neuropathy remains elusive in approximately 20% cases, especially axonal neuropathies. Indications for nerve biopsy 1. Vasculitic neuropathy: Nerve biopsy is indicated in vasculitic neuropathy, to establish definitive diagnosis before starting treatment. It has been seen that in vasculitis, nerves are more commonly involved than other readily biopsied structures like skin and muscle, and hence a search for vasculitis will have higher yield with a nerve biopsy. Peripheral neuropathy is reported in nearly 52-60 % cases with vasculitis 11. 2. Diabetic neuropathy, especially in cases where superimposed CIDP or vasculitis is suspected. 3. Toxic neuropathies (amiodarone) 4. Infections (HIV, Leprosy) 5. CIDP, and paraproteinemic neuropathies (deposits of IgM in the nerve usually precede IgM gammopathy in serum) 6. Amyloidosis. 7. Hereditary neuropathies with negative appropriate genetic tests. Nerve biopsy can also be helpful by identification of characteristic features, thereafter planning appropriate genetic tests, for example MPZ gene mutations with failure of myelin compaction, MTMR2 gene mutations with numerous myelin 5 outfoldings, or MFN2 mutations with abnormalities of intraaxonal mitochondria. 8. Diagnostic etiology of neuropathy is not established even after a detailed investigation. In a study of 365 nerve biopsies studied in patients with undetermined etiology, Shin J. Oh etal found clinically relevant information or helpful information in nearly 45 % cases. In the same study, specific diagnosis was reached in 24 % of cases. A diagnosis of vasculitis was established in 12 % cases, making it the most common diagnosis among those with specific diagnosis 12. Selection of nerve for biopsy Selection of nerve for biopsy including sural nerve, superficial peroneal nerve, superficial branch of radial nerve, dorsal cutaneous branch of ulnar nerve is done depending on the clinical scenario. Biopsy should preferably be obtained from a nerve indicating clinical and electrophysiological abnormalities. The nerve to be biopsied may also be indicated by imaging techniques (MRI and ultrasonography) demonstrating affected nerve segments. Sural nerve is usually preferred for biopsy, due to long length of the nerve, pure sensory distribution, and protection behind the lateral malleoli and easily testable electro physiologically. Because of the above mentioned reasons, the yield of sural nerve biopsy is more with only mild sensory loss as a sequalae and free from compression artefacts 12. Superficial peroneal nerve 6 biopsy combined with peroneus brevis muscle biopsy is preferred in cases of vasculitic neuropathy. Studies have demonstrated that such combined muscle and nerve biopsy has a moderately increased yield in demonstrating vasculitis in comparison to nerve biopsy alone 13, 14, however, study by Bennette et al showed no significant increase in yield with combined biopsy compared to nerve biopsy alone 15. Other sensory nerves such as the superficial femoral, superficial radial and the antebrachial cutaneous nerves may also be
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