Amyotrophic lateral sclerosis care and research in the United States during the COVID-19 pandemic: Challenges and opportunities.

Coronavirus disease 2019 has created unprecedented challenges for amyotrophic lateral sclerosis (ALS) clinical care and research in the United States. Traditional evaluations for making an ALS diagnosis, measuring progression, and planning interventions rely on in-person visits that may now be unsafe or impossible. Evidence- and experience-based treatment options, such as multidisciplinary team care, feeding tubes, wheelchairs, home health, and hospice, have become more difficult to obtain and in some places are unavailable. In addition, the pandemic has impacted ALS clinical trials by impairing the ability to obtain measurements for trial eligibility, to monitor safety and efficacy outcomes, and to dispense study drug, as these also often rely on in-person visits. We review opportunities for overcoming some of these challenges through telemedicine and novel measurements. These can reoptimize ALS care and research in the current setting and during future events that may limit travel and face-to-face interactions.

Intermuscular coherence as an early biomarker for amyotrophic lateral sclerosis: The protocol for a prospective, multicenter study.

OBJECTIVE: To describe the protocol of a prospective study to test the validity of intermuscular coherence (IMC) as a diagnostic tool and biomarker of upper motor neuron degeneration in amyotrophic lateral sclerosis (ALS). METHODS: This is a multicenter, prospective study. IMC of muscle pairs in the upper and lower limbs is gathered in ∼650 subjects across three groups using surface electrodes and conventional electromyography (EMG) machines. The following subjects will be tested: 1) neurotypical controls; 2) patients with symptomatology suggestive for early ALS but not meeting probable or definite ALS by Awaji Criteria; 3) patients with a known ALS mimic. The recruitment period is between 3/31/2021 and 12/31/2025. Written consent will be sought from the subject or the subject's legally authorized representative during enrollment. RESULTS: The endpoints of this study include: 1) whether adding IMC to the Awaji ALS criteria improve its sensitivity in early ALS and can allow for diagnosis earlier; 2) constructing a database of IMC across different ages, genders, and ethnicities. SIGNIFICANCE: This study may validate a new inexpensive, painless, and widely available tool for the diagnosis of ALS.

Leptomeningeal Disease Secondary to Thr60Ala Transthyretin Amyloidosis: Case Report and Review of the Literature.

A 31-year-old woman with transthyretin (TTR) amyloidosis secondary to a Thr60Ala mutation developed recurrent stroke-like episodes with fluctuating mental status. Evaluation for stroke and seizures was unrevealing. She was found to have leptomeningeal contrast enhancement on magnetic resonance imaging, which was confirmed to be CNS TTR amyloidosis on histopathology following brain and dura biopsy. While leptomeningeal disease has rarely been known to be associated with TTR amyloidosis, this is the first documented case of leptomeningeal disease secondary to a Thr60Ala mutation in the TTR gene. A literature review of TTR amyloidosis is presented with special focus on the treatment of leptomeningeal TTR amyloidosis.

Mononeuropathy Multiplex After COVID-19.

OBJECTIVES: COVID-19 is a novel coronavirus that emerged in 2019 and is responsible for a global pandemic. Numerous neurologic manifestations have been described in the literature regarding COVID-19, but most studies are focused on the central nervous system. The authors have noted an association between prior COVID-19 infection and the development of a systemic neuropathy that manifests with asymmetric sensorimotor loss in the peripheral nervous system. We describe 4 cases of mononeuropathy multiplex that were diagnosed after COVID-19 infection. METHODS: All patients included were treated for severe COVID-19 infection at New York Presbyterian Hospital and subsequently referred to the Columbia Peripheral Neuropathy Center for persistent neuropathy. RESULTS: Patient history, COVID-19 disease course, and mononeuropathy multiplex diagnostic evaluation of the 4 patients are recounted. CONCLUSIONS: We postulate a connection between COVID-19 and the development of mononeuropathy multiplex with implications in prognostication, rehabilitation strategies, and future treatments.

Spinal Cord Compression Secondary to Extramedullary Hematopoiesis: Case Report and Review of the Literature.

Extramedullary hematopoiesis (EMH) is a rare cause of spinal cord compression (SCC). EMH represents the growth of blood cells outside of the bone marrow and occurs in a variety of hematologic illnesses, including various types of anemia and myeloproliferative disorders. Although EMH usually occurs in the liver, spleen, and lymph nodes, it may also occur within the spinal canal. When this occurs, the mass effect can compress the spinal cord, potentially leading to the development of neurological deficits. We present a case of SCC secondary to EMH. This report illustrates the importance of considering EMH in the differential diagnosis of SCC, even in the absence of signs of its most common etiologies.

Delayed Tension Pneumocephalus following Gunshot Wound to the Head: A Case Report and Review of the Literature.

Tension pneumocephalus is a rare complication of head trauma and neurosurgical procedures, amongst other causes. It is defined by the combination of intracranial air, increased intracranial pressure, and mass effect. Although it often presents soon after surgery, it can also rarely present in a delayed fashion. We present a case of delayed tension pneumocephalus, occurring approximately 16 weeks after bifrontal craniectomy for a self-inflicted gunshot wound. Following a month of rhinorrhea, postnasal drip, and cough, the patient presented with a sensation of expansion in the area of the right forehead. As tension pneumocephalus is an emergency that can be fatal, this patient was treated expediently and avoided severe neurological deficits. The case recounted here is important as a demonstrative example that tension pneumocephalus does not always follow a defined course immediately after trauma or neurosurgery but rather can develop insidiously without obvious signs.

Metastatic Mantle Cell Lymphoma to the Pituitary Gland: Case Report and Literature Review.

We present an unusual case of a metastatic mantle cell lymphoma (MCL) to the pituitary gland. The patient had a known history of MCL for which she previously received chemotherapy. She presented with new-onset diplopia and confusion, and reported a history of progressive vision blurriness associated with headache, nausea, and vomiting. MRI of the brain showed an enhancing lesion within the sella turcica involving the cavernous sinuses bilaterally, extending into Meckel's cave on the left, and abutting the optic nerves bilaterally. Following surgical excision, histopathology revealed the tumor to be a MCL. Metastatic pituitary tumors are rare and have been estimated to make up 1% of tumors discovered in the sellar region. The two most common secondary metastatic lesions to the sella are breast and lung carcinoma followed by prostate, renal cell, and gastrointestinal carcinoma. Metastatic lymphoma to the pituitary gland is especially rare and is estimated to constitute 0.5% of all metastatic tumors to the sella turcica. To our knowledge, this is the first reported case of MCL metastasizing to the pituitary gland.