Title : A Case of vasculitic neuropathy presenting with progressive sensory motor limb involvement, posterior reversible encephalopathy syndrome and systemic symptoms in a 70 year old woman
Abstract:
A 70-year-old woman was brought to the Emergency department after developing progressive weakness and altered sensation in both her hands and feet. She reported being unable to bear weight ,episode of urinary incontinence, gradual decline in her health over the past nine weeks also marked by unintentional weight loss of approximately one stone, occasional nausea and vomiting. Family also reported intermittent confusion. She also had history of cervical spinal stenosis , Raynaud's phenomenon osteoarthritis, fibromyalgia, and rheumatoid arthritis.
On initial assessment, her hemoglobin was 75 g/L. was diagnosed with iron deficiency anemia .Upper GI endoscopy revealed Esophagitis which was treated with high dose PPI. MRI of the spine showed multilevel degenerative spondylotic changes in the cervical and lumbar spine, with disc herniations at several levels. A posterior disc herniation at C4/C5 caused indentation on the thecal sac and the anterior aspect of the cord with mild foraminal narrowing. Importantly, there was no evidence of acute spinal canal stenosis, cord or cauda equina compression, or myelopathy.
The following day, her condition suddenly deteriorated. She developed status epilepticus and her blood pressure at the time was documented at 200/80 mmHg and was transfer to intensive care for management of status epilepticus.
Initially was empirically treated with ceftriaxone, amoxicillin, and acyclovir for Meningoencephalitis and above was treatment stopped once Cerebrospinal fluid analysis ruled out bacterial and viral causes.
A CT venogram of the head ruled out venous sinus thrombosis . A non-contrast CT head followed by MRI head revealed radiological features suspicious for posterior reversible encephalopathy syndrome (PRES)
Echocardiography demonstrated preserved left ventricular systolic function, left pleural effusion, and a trivial pericardial effusion without hemodynamic compromise.
Differential diagnoses, including paraneoplastic syndromes, systemic vasculitides, and lupus, were considered. Serological investigations, including antinuclear antibodies (ANA) and ANA profile, were negative.
A PET-CT was performed, revealing a highly fluorodeoxyglucose (FDG)-avid right vocal cord nodule, FDG-avid mediastinal and porta hepatis lymph nodes .ENT suggested vocal cotf nodule is benign. patient declined lymph node biopsy at that stage. NCS demonstrated an asymmetric neuropathy with left radial palsy, bilateral ulnar palsy, and possible bilateral peroneal involvement. Electromyography showed severe subacute denervation and a generalized axonal neuropathy, highly suggestive of vasculitis neuropathy. Systemic features--weight loss, fluctuating confusion, inflammatory arthritis, Raynaud's phenomenon, and pleural and pericardial involvement--all raised suspicion for systemic vasculitis.
Sural nerve biopsy revealed reduced axonal density and striking perineuritis with evidence of both healed and active vasculitis. These findings confirmed the diagnosis of vasculitis neuropathy. Patient was commenced on high-dose prednisolone (60 mg daily) which halted progression of motor and sensory weakness with initial mild improvement.
