Anant M. Shenoy

Guidelines in practice: treatment of painful diabetic neuropathy.

This article describes a patient with a painful diabetic peripheral neuropathy. Features of his history, examination, and diagnostic workup are presented. His treatment course is described as guided by the AANs evidence-based guideline on the treatment of painful diabetic neuropathy. Lastly, features of coding for diabetic peripheral neuropathy are reviewed.

Muscle-Eye-Brain disease.

A term female infant was evaluated for global developmental delay, hypotonia, hyporeflexia, diffuse weakness including facial muscles, and visual impairment with optic nerve hypoplasia. In the absence of family history or perinatal concerns, an extensive investigation was performed, including lab studies, muscle biopsy, brain MRI and focused genetic testing. This revealed elevated serum CK, a structurally abnormal brain, and a dystrophic-appearing muscle biopsy with evidence of a glycosylation defect in the alpha-dystroglycan complex. Of the 6 known related genes, testing of the POMGnT1 gene showed three heterozygous missense mutations. Thus her history, examination, biopsy specimen, imaging, laboratory, and genetic studies are all consistent with the diagnosis of Muscle-Eye-Brain (MEB) disease. MEB is one of an emerging spectrum of congenital disorders that involve both central and peripheral nervous systems, described further in this case report.

Fahr’s disease

A 54 year-old man with a history of bipolar disorder presented to the neurology clinic with a two-year history of tremor affecting both hands that was worse on action. He also complained of being “slow moving” for many years. There was no family history of tremor or other movement disorders. On examination, his verbal responses were slow and he had impaired short-term memory. Glabellar …

Saphenous mononeuropathy after popliteal vein aneurysm repair.

Introduction:Saphenous mononeuropathy has been a well recognized consequence of lower extremity surgery. However, this complication has not been previously described with popliteal vein aneurysm repair. Case Presentation:We report the case of a 42-year-old woman with a saphenous mononeuropathy after popliteal vein aneurysm repair. Her saphenous neuropathy was confirmed by nerve conduction studies. Her case gives us an opportunity to review saphenous mononeuropathy and its many different etiologies. We also review the role of electrodiagnostic studies in the diagnosis of saphenous mononeuropathy. Conclusions:Though this particular iatrogenic injury has not previously been described, both neurologists and surgeons should be aware of this complication following popliteal vein aneurysm resection with saphenous vein interposition.

Novel SPG3A and SPG4 mutations in two patients with Silver syndrome.

Hereditary spastic paraplegia encompasses a group of disorders that are characterized by progressive lower extremity weakness and spasticity. We describe two patients with Silver phenotype including one with a novel SPG4 (Spastin) mutation and a second with a known SPG 4 mutation (previously unassociated with this phenotype) and a concomitant previously unreported mutation in SPG3A (Atlastin). These cases suggest that Silver syndrome may be associated with a wider variety of genotypes than previously described.

Quality improvement in neurology: Stroke and Stroke Rehabilitation Quality Measurement Set update

Stroke is the fifth leading cause of death in the United States and the second leading cause of disability worldwide.1,2 “On average, every 40 seconds, someone in the United States has a stroke, and someone dies of one approximately every 4 minutes.”3 Each year, nearly 800,000 people experience stroke, with up to 185,000 experiencing recurrent stroke events.3 Strokes occur at any age, and risk increases with age.4 Nearly half of older stroke survivors experience moderate to severe disability.5

An Asymmetric Sensory Polyneuropathy Related to Thiamine Deficiency From a Gluten-free Diet

nervous system (CNS) through peripheral nerves. Recent evidence suggests that axonal transport mediates WNV entry into the CNS and induces acute flaccid paralysis. This patient presented as an acute aggressive paraparesis severe enough to impede pulmonary function. Extensive workup was notable for high WNV antibody titers in the CSF and serum while remaining negative for other infectious and autoimmune causes. Magnetic resonance imaging showed subtle enhancement only of the nerve roots, whereas electrodiagnostic studies were consistent with a MADSAM syndrome. WNV infection can cause protean manifestations in the CNS; a polio-like direct infection of the anterior horn cells is most common but other manifestations can include acute meningoencephalitis. A prior case series of electrodiagnostic studies done during the acute infective stage of WNV patients showed normal SNAPs and normal or only mildly reduced CMAPs without slowed conduction velocity, similar to our patient’s initial presentation; however, a repeat study done after the acute infective stage in these patients was not reported. Our patient displayed pronounced asymmetric sensory involvement as can be seen in MADSAM later in the course. The evolution of electrodiagnostic profiles in WNV infections has not been published, and given the varied manifestations of acute infection, may be widely varied as well. This case is particularly illustrative in that it involves a patient who initially presented with symptoms of MADSAM syndrome and was later found to have WNV antibodies. Thus, it gives us an insight into the natural history of a WNV CNS infection, beginning as an inflammatory demyelinating polyneuropathy and continuing as prolonged motor and sensory neuropathy.

Guidelines in practice: therapies for benign paroxysmal positional vertigo.

Benign paroxysmal positional vertigo (BPPV) is the most common cause of vertigo. This article describes the presentation and physical examination of a patient with BPPV. The case is then used to explore various features of the diagnosis, examination, and management of BPPV, with a focus on the 2008 AAN practice parameter on therapies for BPPV.

Quality Improvement in Neurology: Neurotology Quality Measurement Set:

Quality Improvement in Neurology: Neurotology Quality Measurement Set Otolaryngology– Head and Neck Surgery 2018, Vol. 159(4) 603–607 American Academy of Otolaryngology–Head and Neck Surgery Foundation and American Academy of Neurology Institute 2018 Reprints and permission: sagepub.com/journalsPermissions.nav DOI: 10.1177/0194599818790947 http://otojournal.org Habib Rizk, MD, MSc, Yuri Agrawal, MD, MPH, Susan Barthel, Marc L. Bennett, MD, MMHC, Joni K. Doherty, MD, PhD, Patricia Gerend, Daniel R. Gold, DO, David Morrill, John G. Oas, MD, J. Kirk Roberts, MD, Erika Woodson, MD, David A. Zapala, PhD, Amy Bennett, JD, and Anant M. Shenoy, MD

Turning Electromyography Reports Upside Down: A Pilot Study Surveying Referring Providers

Providers are expressing a desire for more efficient ways to retrieve relevant clinical data from the Electronic Health Record. In an effort to improve our Electromyography and Nerve Conduction Study reports, we surveyed referring providers on the effects of having the IMPRESSION at the start of the report. Our survey respondents felt that using this format for an Electromyography and Nerve Conduction Study report significantly improved the quality of the report while saving them time and/or mouse clicks when interpreting the report. Electro diagnosticians might consider using this format for their Electromyography and Nerve Conduction Study reports to improve referring provider satisfaction.