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A 40-year-old woman presented to the neuromuscular clinic for evaluation of chronic right shoulder weakness of 1-year duration. During that time, she noticed progressive difficulties lifting her right arm overhead and reaching for objects on the back seat of her car. Around 3 months after the onset of shoulder weakness, she started to develop an intermittent achy sensation behind the right ear and hoarseness of her voice. She noted difficulty talking over loud noises and in phone conversations. She denied vision, taste, or hearing changes, dysphagia, dyspnea, numbness, or weakness in other limbs.

Background:In January 2020, the first case of Guillain Barre syndrome (GBS) due to COVID-19 was documented in China. GBS is known to be postinfectious following several types of infections. Although causality can only be proven through large epidemiological studies, we intended to study this association by a thorough review of the literature.Methods:We searched PubMed, EMBASE, and Google scholar and included all papers with English or Spanish full text and original data of patients with GBS and recent COVID infection. Variables of interest were demographics, diagnostic investigations, and the latency between arboviral and neurological symptoms. Further variables were pooled to identify GBS clinical and electrophysiological variants, used treatments, and outcomes. The certainty of GBS diagnosis was verified using Brighton criteria.Results:We identified a total of 109 GBS cases. Ninety-nine cases had confirmed COVID-19 infection with an average age of 56.07 years. The average latency period between the arboviral symptoms and neurologic manifestations for confirmed COVID-19 cases was 12.2 d. The predominant GBS clinical and electromyography variants were the classical sensorimotor GBS and acute demyelinating polyneuropathy respectively. Forty cases required intensive care, 33 cases required mechanical ventilation, and 6 cases were complicated by death.Conclusions:Studies on COVID-19-related GBS commonly reported sensorimotor demyelinating GBS with frequent facial palsy. The time between the onset of infectious and neurological symptoms suggests a postinfectious mechanism. Early diagnosis of GBS in COVID-19 patients is important as it might be associated with a severe disease course requiring intensive care and mechanical ventilation.

We report a 73-year-old man with Val142Ile transthyretin (TTR) amyloidosis and an atypical clinical presentation of upper-extremity-predominant neuropathy without significant autonomic or cardiac involvement. TTR familial amyloid polyneuropathy commonly presents as length-dependent sensorimotor polyneuropathy with marked and early autonomic involvement. Multiple pathogenic mutations in TTR gene have been identified, of which Val50Met is commonly associated with TTR familial amyloid polyneuropathy, and Val142Ile is commonly associated with familial amyloid cardiomyopathy. Our patient is from a nonendemic region, without family history for amyloidosis. Predominant upper-extremity neuropathy, without significant cardiac or autonomic involvement, distinguishes this case from previously reported Val142Ile-mutated TTR amyloidosis.

Objectives Mild inflammatory diabetic neuropathies (IDNs) overlap with diabetic sensorimotor neuropathy (DPN) in clinical presentation and electrophysiological and laboratory tests. This study is to determine whether IDN can be differentiated from DPN by clinical features, electrophysiological, pathological, or laboratory tests. Methods Suspected IDN cases were identified by a subacute onset and progressive sensory or motor neuropathy in patients with diabetes. Results IDN occurred earlier in the course of diabetes mellitus and had higher prevalence of limb weakness, walking difficulty, and more severe electrophysiological abnormalities suggesting both demyelination and axonal loss. Sensory nerve biopsies in IDN showed perivascular inflammatory infiltrates, decreased fiber density, increased demyelination, and axonal degeneration. Most patients with IDN improved with immunotherapy. Conclusions Features that favor IDN over DPN are limb weakness, more severe nerve conduction abnormalities, inflammatory infiltrates on nerve biopsy, and a favorable response to immunotherapy. A nerve biopsy can help establish an inflammatory cause.

Rationale: Sensory neuronopathy can be a devastating peripheral nervous system disorder. Profound loss in joint position is associated with sensory ataxia, and reflects degeneration of large-sized dorsal root ganglia. Prompt recognition of sensory neuronopathies may constitute a therapeutic window to intervene before there are irreversible deficits. However, nerve-conduction studies may be unrevealing early in the disease course. In such cases, the appearance of dorsal column lesions on spinal-cord MRI can help in the diagnosis. However, most studies have not defined whether such dorsal column lesions may occur within earlier as well as chronic stages of sensory neuronopathies, and whether serial MRI studies can be used to help assess treatment efficacy. In this case-series of three sensory neuronopathy patients, we report clinical characteristics, immunological markers, nerve-conduction and skin-biopsy studies, and neuroimaging features. Patient concerns: All three patients presented with characteristic features of sensory neuronopathy with abnormal spinal-cord MRI studies. Radiographic findings included non-enhancing lesions in the dorsal columns that were longitudinally extensive (spanning ≥ 3 vertebral segments). Diagnoses: All patients had anti-Ro/SS-A and/or anti-La/SS-B antibodies, with patients one and two having Sjögren's syndrome. MRI findings were similar when performed in the earlier stages of a sensory neuronopathy (patient one, after four months) and chronic stages (patients two and three, after five and three years, respectively). Interventions: Patient one was treated with rituximab combined with intravenous immunoglobulin therapy. Outcomes: Patient one was initially wheelchair-bound and had improved ambulation after treatment. In this patient, serial MRI studies revealed partial resolution of dorsal column lesions, associated with decreased sensory ataxia and improved nerve-conduction studies. Lessons: In addition to vitamin B12 and copper deficiency, it is important to include sensory neuronopathies in the differential diagnosis of dorsal column lesions. MRI spinal-cord lesions have similar appearances in the earlier as well as chronic phases of a sensory neuronopathy, and therefore suggest that such dorsal column lesions may reflect inflammatory as well as a gliotic burden of injury. MRI may also be a useful longitudinal indicator of treatment response.

Objective: We describe a patient with diabetic amyotrophy (radiculoplexopathy) that presented initially as isolated L5 radiculopathy. Background Diabetic amyotrophy is characterized by severe pain followed by weakness and wasting of pelvifemoral muscles, either unilaterally or bilaterally. It commonly affects L2-L4 roots, with the femoral and obturator nerves being mostly affected. Design/Methods: A 62 year old with history of diabetes mellitus-II presented with 2 weeks history of right foot drop and back pain radiating down into right lateral thigh, leg and dorsum of the foot. Examination revealed 0/5 right dorsiflexion and 0/5 toe extension, 2/5 inversion, 4/5 eversion and normal plantar flexion. Over the next 3 months the pain worsened and weakness progressed to involve the right thigh and then the left thigh leading to an asymmetrical (right worse than left) flaccid paraparesis with areflexia in the legs, and sensory loss affecting mostly the medial thighs. She had a severe weight loss (around 30-40Lb.) during that period. Extensive workup for malignancy and vasculitis was negative. MRI of the spine revealed mild enhancement in the L5 and S1 roots. CSF analysis showed a hyperproteinorraquia of 96 mg/dL. Over the next year, she showed slow improvement but is still non-ambulatory. Results: Initial EMG done 3 weeks after onset of foot drop revealed an absent right superficial peroneal SNAP and active denervation in the L5 innervated muscles including the lumbar paraspinal muscles, consistent with an L5 radiculopathy. Repeat EMG 2 months later showed severe bilateral and asymmetric lumbosacral polyradiculopathies, affecting L3-4 roots bilaterally and right L5 root with prominent active denervation. The findings on EMG were consistent with diabetic amyotrophy (radiculoplexopathy). Conclusions: Diabetic Amyotrophy may atypically present with unilateral L5 radiculopathy that ultimately extends into the upper lumbar roots. Disclosure: Dr. Piccione has nothing to disclose. Dr. Farheen has nothing to disclose. Dr. Katirji has nothing to disclose.