|Year : 2020 | Volume
| Issue : 5 | Page : 562-563
Nonsystemic vasculitic neuropathy
Shalesh Rohatgi, Dhaval Dave, Furqan Khan
Department of Neurology, Dr. D.Y. Patil Medical College and Hospital, Dr. D.Y. Patil Vidyapeeth, Pimpri, Pune, Maharashtra, India
|Date of Submission||08-Jun-2019|
|Date of Decision||01-Nov-2019|
|Date of Acceptance||06-Jan-2020|
|Date of Web Publication||7-Sep-2020|
701, MIDC Staff Quarters, Dr. D. Y. Patil Medical College Campus, Sant-Tukaram Nagar, Pimpri, Pune - 411 018, Maharashtra
Source of Support: None, Conflict of Interest: None
A 21-year-old female born of nonconsanguineous marriage presented with gradually progressive quadriplegia for 3 years. Weakness was distal more than proximal, with wasting. She also had moderate pain in extremities. She had significant weight loss. Clinically, she had lower motor neuron flaccid quadriplegia with absent deep tendon jerks. Sensations of touch and pain were diminished in bilateral feet till ankle joints. Peripheral nerves were not thickened. Nerve conduction study was suggestive of pure axonal sensorimotor polyneuropathy. All routine hematological and biochemical parameters, erythrocyte sedimentation rate, antinuclear antibodies, and antinuclear cytoplasmic antibodies were negative. Cerebrospinal fluid showed no cells, normal sugar, and raised proteins. Biopsy of sural nerve was suggestive of vasculitic neuropathy. This was a case of nonsystemic vasculitic neuropathy that responded to steroids.
Keywords: Nonsystemic vasculitic neuropathy, sensorimotor polyneuropathy, vasculitic neuropathy
|How to cite this article:|
Rohatgi S, Dave D, Khan F. Nonsystemic vasculitic neuropathy. Med J DY Patil Vidyapeeth 2020;13:562-3
| Introduction|| |
Nonsystemic vasculitic neuropathy (NSVN) is a rare autoimmune disorder restricted to only the peripheral nervous system, and diagnosis is made only with nerve biopsy. Peripheral neuropathy due to vasculitis without manifestations of disorders in other systems was first reported by Kernohan and Woltman in 1938. NSVN is seen in one-third of all cases and involves small- and medium-sized arteries. There is no clinical evidence of other organ involvement, and tests for vasculitis are negative. Three patterns of clinical involvement in NSVN have been identified: multifocal neuropathy, asymmetric polyneuropathy (also commonly known as overlapping multifocal neuropathy), and distal symmetric polyneuropathy. Neuropathies are painful in >80% of patients. Cerebrospinal fluid may show increased proteins. 28% of patients lose weight and 13% develop fever. Most patients follow a stepwise or relapsing clinical course, but the disease steadily progresses in 40%. The diagnostic gold standard for NSVN is vessel wall inflammation and damage identified by nerve biopsy; a probable diagnosis is possible if the biopsy findings are suspicious but not pathognomonic. Cranial nerve involvement occurs in 8% of patients, typically involving cranial nerve VII.
| Case Report|| |
We report a case of a 21-year-old female, born of nonconsanguineous marriage who presented with a history of gradually progressive weakness in all four limbs, more in lower limbs than upper limbs, more distal, with dull-aching pain, and wasting of distal extremities for 3 years. The patient was confined to bed and could not get up without support. Her weight was 30 kg with a body mass index of 13.1 kg/m2. She had wasting in all four limbs with pes-cavus deformity, hypotonia in all four limbs. Power in the upper limbs was grade 4 at shoulders, grade 3 at wrist, handgrip was 70% bilaterally. In lower limbs, grade 2 at hip and grade 1 at ankle bilaterally. All deep tendon jerks were absent. There was impairment of touch, pain, and temperature below ankle joints bilaterally. Superficial plantar reflexes were not elicitable. Peripheral nerves were not thickened.
Nerve conduction study was suggestive of pure axonal sensorimotor polyneuropathy. All routine hematological and biochemical investigations, erythrocyte sedimentation rate, antinuclear antibodies, and antinuclear cytoplasmic antibodies were negative. CSF analysis showed no cells, sugar of 50 mg% (blood sugar 96 mg%), and proteins of 104 mg%. Serum protein electrophoresis was suggestive of polyclonal hypergammaglobulinemia; M band was not detected.
Sural nerve biopsy showed prominent acute axonal degeneration; myelinated fiber loss was severe (small more than large) with occasional regenerating clusters. Epineural vessels were thickened with luminal occlusion in one of the arterioles. Few perivascular lymphocytes were seen. Pearls stain for hemosiderin was negative. A diagnosis of NSVN was made and the patient was treated with oral prednisolone 30 mg/day for 6 weeks followed by gradual tapering and then maintenance dose of 10 mg/day. She showed significant recovery.
| Discussion|| |
NSVN is a rare autoimmune disorder restricted to only the peripheral nervous system, and diagnosis is made only with nerve biopsy. Three patterns of clinical involvement in NSVN have been identified: multifocal neuropathy, asymmetric polyneuropathy (also commonly known as overlapping multifocal neuropathy), and distal symmetric polyneuropathy. A large retrospective cohort with extended follow-up of 48 patients (30 women and 18 men) with a median of 63 months of follow-up showed that most patients (85%) had extensive, overlapping involvement of multiple nerves. Only one had a symmetric polyneuropathy. Most neuropathies (96%) were painful. In 96%, nerve damage was distally accentuated, but most had concurrent proximal weakness. Diagnostic sensitivity was 58% for superficial peroneal nerve/peroneus brevis muscle biopsy and 47% for sural nerve biopsy.
In our case also, the patient had symmetric polyneuropathy pattern and was painful too. Distal involvement was more, but the patient also had concurrent proximal weakness. Diagnosis in our case was based on sural nerve biopsy findings.
Corticosteroid monotherapy caused significant improvement in majority of cases. Combination of corticosteroid and cytotoxic therapy was effective in inducing remission and improving disability, with trends toward reduced relapses and chronic pain in nonresponders to corticosteroid monotherapy. Treatment with cyclophosphamide for 6 months decreased the relapse rate, which was 46% for all patients. Disease/treatment-related mortality was 10%. Six percent developed cutaneous involvement. Although chronic pain persisted in 60% of survivors, 80% had good outcomes.
In our case, corticosteroid monotherapy was used and was beneficial in inducing remission and improving disability. The patient was maintained on low-dose corticosteroids and showed significant improvement.
| Conclusion|| |
NSVN most commonly presents as a distally accentuated, painful, sensorimotor polyneuropathy, although other patterns of NSVN do exist. Aside from pain, neurologic prognosis is unexpectedly good with corticosteroid monotherapy. Nonresponders require combination corticosteroid and cytotoxic therapy and results are good.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
| References|| |
Collins MP, Hadden RD. The nonsystemic vasculitic neuropathies. Nat Rev Neurol 2017;13:302-16.
Kernohan JW, Woltman HW. Periarteritis nodosa: A clinicopathologic study with special reference to the nervous system. Arch Neurol Psychiatry 1938;39:655-86.
Collins MP, Dyck PJ, Gronseth GS, Guillevin L, Hadden RD, Heuss D, et al
. Peripheral Nerve Society Guideline on the classification, diagnosis, investigation, and immunosuppressive therapy of non-systemic vasculitic neuropathy: Executive summary. J Peripher Nerv Syst 2010;15:176-84.
Collins MP, Mendell JR, Periquet MI, Sahenk Z, Amato AA, Gronseth GS, et al
. Superficial peroneal nerve/peroneus brevis muscle biopsy in vasculitic neuropathy. Neurology 2000;55:636-43.
Collins MP, Periquet MI, Mendell JR, Sahenk Z, Nagaraja HN, Kissel JT, et al.
Nonsystemic vasculitic neuropathy: Insights from a clinical cohort. Neurology 2003;61:623-30.