Aiesha Ahmed

Isaacs syndrome: A review

Isaacs syndrome is a peripheral nerve hyperexcitability (PNH) syndrome that presents as continuous motor activity. Clinical findings include cramps, fasciculations, and myokymia. Electrodiagnosis plays a key role in diagnosis by demonstrating after‐discharges on nerve conduction studies, and fasciculation potentials, myokymic discharges, neuromyotonic discharges, and other types of abnormal spontaneous activity on needle examination. Etiopathogenesis involves the interaction of genetic, autoimmune, and paraneoplastic factors, which requires a broad‐ranging evaluation for underlying causes. Initial treatment is symptomatic, but immune therapy is often needed and can be effective. The purpose of this review is to describe the syndrome and its pathogenesis, assist the reader in evaluating patients with suspected Isaacs syndrome and distinguishing it from other disorders of PNH, and suggest an approach to management, including both symptomatic and immunomodulating therapy. Muscle Nerve 52: 5–12, 2015

Amyotrophic Lateral Sclerosis: What Role Does Environment Play?

The cause of sporadic amyotrophic lateral sclerosis (ALS) is not known. Studies associate toxic, dietary, infectious, neoplastic, and physical factors as underlying, predisposing, or pathogenic influences. Historical, conventional, and novel disease mechanisms, acting solely or in concert, convert previously healthy individuals into terminally ill patients. Despite intensive investigations in the previous decades, the underlying cause and effective treatments elude researchers. Discovering causative mechanisms in sporadic ALS will facilitate effective treatments and cures for this disorder. After a brief review of the disease process itself, this article discusses potential environmental influences on the development of ALS.

Pseudobulbar affect: prevalence and management

Pseudobulbar affect (PBA) may occur in association with a variety of neurological diseases, and so may be encountered in the setting of amyotrophic lateral sclerosis, extrapyramidal and cerebellar disorders, multiple sclerosis, traumatic brain injury, Alzheimer’s disease, stroke, and brain tumors. The psychological consequences and the impact on social interactions may be substantial. Although it is most commonly misidentified as a mood disorder, particularly depression or a bipolar disorder, there are characteristic features that can be recognized clinically or assessed by validated scales, resulting in accurate identification of PBA, and thus permitting proper management and treatment. Mechanistically, PBA is a disinhibition syndrome in which pathways involving serotonin and glutamate are disrupted. This knowledge has permitted effective treatment for many years with antidepressants, particularly tricyclic antidepressants and selective serotonin reuptake inhibitors. A recent therapeutic breakthrough occurred with the approval by the Food and Drug Administration of a dextromethorphan/quinidine combination as being safe and effective for treatment of PBA. Side effect profiles and contraindications differ for the various treatment options, and the clinician must be familiar with these when choosing the best therapy for an individual, particularly elderly patients and those with multiple comorbidities and concomitant medications.

Recovery of neurologic dysfunction with early intervention of vitamin B12.

A patient developed numbness and tingling in distal extremities with subsequent weakness. Evaluation revealed B12 deficiency. She had evidence of myelopathy on imaging studies and polyneuropathy on electrodiagnostic testing. Treatment with B12 caused remittance of symptoms and resolution/improvement of abnormalities found on the imaging and electrodiagnostic studies. This case demonstrates that early intervention with B12 supplementation can cause reversal of both central and peripheral nervous system dysfunction.

Rehabilitation of brachial plexus and peripheral nerve disorders

Peripheral nerve lesions are common and can present in a variety of ways. Peripheral nerve injury can result from a broad spectrum of causes. For the majority of patients, rehabilitation is generally indicated regardless of etiology. Evaluation and treatment by a multidisciplinary team including neurologists, psychiatrists, surgeons, occupational and physical therapists, and therapists with specialized training in orthotics maximizes the potential for recovery. This chapter will focus on those upper and lower extremity neuropathies that are most commonly seen in clinical practice. In addition, we discuss various rehabilitative strategies designed to improve function and quality of life.

Pyridoxine Toxicity Small Fiber Neuropathy with Dysautonomia: A Case Report

Pyridoxine (vitamin B6) toxicity is a well-known cause of primary sensory, length-dependent, axonal polyneuropathy. Although sensory symptoms predominate, autonomic symptoms have also been reported in some cases. To date, there is no objective evidence of autonomic dysfunction reported in the literature. We present the case of a 41-year-old woman with 2 years of progressive burning pain, numbness, tingling, and weakness in a stocking-glove distribution who was found to have severe pyridoxine toxicity. Concurrent presence of large and small fiber nerve dysfunction was noted in the form of abnormal electromyography/nerve conduction study demonstrating a chronic sensory polyneuropathy and autonomic testing demonstrating abnormal responses to quantitative sweat testing and cardiovagal function testing. This case highlights the need for consideration of small fiber nerve damage by obtaining autonomic testing in cases of pyridoxine toxicity.

Advanced Practice Clinician Training for Neurology

Background The specialty of Neurology is faced with a fundamental problem of economics: supply and demand. The projected increase in provider supply is unlikely to keep up with projected increases in patient-care demand. Many large academic centers have used residents to meet this patient-care demand. However, the conflict between education of residents and patient-care needs has created a hindrance to both of those missions. Many specialties have been using advanced practice clinicians (APCs) to help address the need for patient care. In the setting of a residency program, this availability of APCs can help to alleviate patient-care demands for the resident and allow for better allocated educational time. Neurology has not historically been a popular choice for APCs and a standardized educational curriculum for a Neurology APC has not been established. Methods The authors share an example curriculum recently implemented for training new inpatient Neurology APCs. This curriculum includes a 12-week program complete with rotations through various subspecialties and proposes fundamental lecture topics for use in education. The authors share their expectations for clinical duties that evolve over the course of the 12-week program in conjunction with expectations for increasing clinical knowledge as well as efficiency in system utilization. Conclusion The addition of APCs to support a busy inpatient Neurology practice has obvious beneficial implications but the integration and education of this new staff must be structured and well-designed to support the confidence of the APC in both their knowledge and their role as an indispensable member of the care team.

Meralgia Paresthetica and Femoral Acetabular Impingement: A Possible Association

Meralgia paresthetica consists of pain and dysesthesia in the anterolateral thigh. Etiology is divided into spontaneous and iatrogenic causes. To my knowledge this has never been attributed to femoral acetabular impingement. This case highlights the presence of lateral femoral cutaneous neuropathy in the setting of femoral acetabular impingement syndrome thus raising the possibility of an association. Keywords Femoral acetabular impingement; Lateral femoral cutaneous nerve; Dysesthesia; Nerve conduction studies

A Case of Dropped Head plus Syndrome

A patient developed neck extensor weakness along with symmetrical, mild proximal extremity weakness. Electrodiagnostic findings were minimal. Subsequently muscle biopsy was undertaken which showed myopathic changes as well as rimmed vacuoles. This case highlights the discussion of histopatholgical findings seen in neck extensor weakness when it occurs in isolation as well as when it is associated with weakness in other distribution. Keywords Neck extensor weakness; Rimmed vacuoles; Myopathy

Neurosyphilis: mighty imitator forays with benign presentation and unique neuroimaging findings

Background Common causes of temporal lobe hyper intensities are central nervous system infections like herpes simplex encephalitis, Lyme disease, limbic encephalitis and vascular pathology like Cerebral Autosomal Dominant Arteriopathy with Subcortical infarcts and Leukoencephalopathy. METHODS Personal assessment, laboratory data analysis and neuroimaging for the patient who was admitted to a central Pennsylvania tertiary care referral centre were conducted. RESULTS A 52-year-old male presented with a 1-year history of diffuse dysesthesia in upper and lower extremities with associated intermittent headaches and neck stiffness. Evaluation with lumbar puncture revealed increased nucleated cells (50ul) with lymphocytic predominance (96%) and an elevated protein level of 109mg/dl. Magnetic resonance imaging (MRI) of the brain showed T2/FLAIR hyper intensity in bilateral subcortical temporal white matter, left-greater-than-right and associated volume loss in cerebral parenchyma. Additional abnormal work up included reactive serum reactive plasma regain and Treponema pallidum antibody particle agglutination. Diagnosis of neurosyphilis was made and the patient was treated with intramuscular (IM) penicillin for 3 weeks. At the time of discharge, his headache and neck stiffness resolved and dysesthesias were decreased in intensity. CONCLUSIONS The diagnosis of neurosyphilis is intricate, and no reference standard exists. Neuroimaging findings of neurosyphilis commonly are cerebral infarctions, leptomeningeal enhancement or non-specific white matter lesions. Less common features on fluid-attenuated inversion recovery (FLAIR) sequences are cortical atrophy and mesial temporal parenchymal signal changes. It is prudent to keep neurosyphilis in differential of mesial temporal lobe white matter changes, as early diagnosis and treatment results in better prognosis.