Leprous Neuropathy: Observational Study Highlighting the Role of Electrophysiology in Early Diagnosis

Background Worldwide leprosy is a common cause of peripheral neuropathy. Electrophysiology is underutilized in its diagnosis. Objective This study aims to evaluate the usefulness of electrophysiological study in the diagnosis of leprous neuropathy. Materials and Methods Clinical and electrophysiological abnormalities of 36 histopathology proven leprosy patients from January 2015 to January 2017 were studied. Statistical Analysis Proportions were compared by Chi-square test. Results Total patients were 36. Thirty-four patients had abnormal electrophysiology and 34 had neurological deficits like weakness, sensory changes, and thickening. By clinical examination, multiple nerve involvement (motor weakness, sensory changes, and nerve thickening) occurred in 29, single nerve in 5, and no nerve involvement in 2. With electrophysiology, multiple nerve involvement (mononeuritis multiplex) was present in 32, single nerve in 2, and normal conduction parameters in 2. From the 36 patients, a total of 1,008 nerves were subjected to clinical examination and 132 were picked up clinically as affected, (13.1%). Electrophysiological study was done in 504 nerves, and 215 were found to be involved, (43%). Nerve abnormality detected by electrophysiology is significantly higher than clinical detection. (Chi-square =164.4054; p = 0.0000). Clinically, the most commonly affected nerve was unar (27) and the least affected was median (2) nerve. Electrophysiology detected 69% of nerves with demyelination and 35% of nerves with axonal features (mosaic pattern). Discussion There was subclinical neuropathy with electroclinical dissociation, as evidenced by more abnormality in electrophysiology than clinical examination. The nerve involvement was mononeuritis or mononeuritis multiplex pattern, both clinically and electrophysiologically. Electrophysiology showed both axonal and demyelinating nerve involvement (mosaic pattern). All the three features are present in leprous neuropathy. In corollary, if a patient has these electrophysiological features, he should be thoroughly investigated for leprosy. Conclusion Triple findings, such as subclinical neuropathy with electroclinical dissociation, mononeuritis multiplex, and mosaic pattern of demyelination and axonopathy, suggest leprous neuropathy

CIDP Masquerading as Mononeuritis Multiplex: The Value of MR Neurography

Background: We present two patients with the Lewis-Sumner variant of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), charting the diagnostic challenge posed by their clinical presentation and electrodiagnostic findings. The learning points center on the use of magnetic resonance imaging (MRI) in establishing a definitive diagnosis when clinical and neurophysiology data do not otherwise add up. Cases: The first patient presented with slowly progressive asymmetric distal weakness of the lower limbs with wasting, weakness, areflexia and numbness on examination. The second patient experienced stepwise asymmetric hand/forearm weakness with deformity and areflexia, plus mild distal sensory impairment. Neurophysiological studies for both patients were initially most suggestive of mononeuritis multiplex, with no evidence of demyelination. Conclusion: The possibility of asymmetric or multifocal CIDP, the Lewis-Sumner variant, should not be forgotten in suspected mononeuritis multiplex and the value of MRI in such cases is discussed.

Neurological manifestations of polyarteritis nodosa: a tour of the neuroaxis by case series

Background Heterogenous central nervous system (CNS) neurologic manifestations of polyarteritis nodosa (PAN) are underrecognized. We review three cases of patients with PAN that illustrate a range of nervous system pathology, including the classical mononeuritis multiplex as well as uncommon brain and spinal cord vascular manifestations. Case presentation Case 1 presented with mononeuritis multiplex and characteristic skin findings. Case 2 presented with thunderclap headache and myelopathy due to spinal artery aneurysm rupture. Both patients experienced disease remission upon treatment. Case 3 presented with headache and bulbar symptoms due to partially thrombosed intracranial aneurysms, followed by systemic manifestations related to visceral aneurysms. She demonstrated clinical improvement with treatment, was lost to follow-up, then clinically deteriorated and entered hospice care. Conclusions Although the peripheral manifestations of PAN are well-known, PAN association with CNS neurovascular disease is relatively underappreciated. Clinician awareness of the spectrum of neurologic disease is required to reduce diagnostic delay and promote prompt diagnosis and treatment with immunosuppressants.

Hypocomplementemic urticarial vasculitis syndrome presenting with bilateral scleritis

Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare autoimmune disorder characterised by recurrent urticarial lesions and acquired hypocomplementemia with systemic manifestations. The authors present the case of a 70-year-old man who presented to the ophthalmology clinic with bilateral scleritis and ocular hypertension. He was diagnosed with HUVS after a 6-month period of bilateral scleritis, vestibulitis, significant weight loss, mononeuritis multiplex and recurrent urticarial vasculitis with pronounced persistent hypocomplementemia and the presence of anti-C1q antibodies. Disease control was eventually obtained with mycophenolate and prednisolone.