Browsing by Author "Morena, Jonathan"

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    Characteristics of Triple Seronegative Myasthenia Gravis: A Single Center Experience
    (RRNMF Neuromuscular Journal) Morena, Jonathan; Jiang, Benjamin; Freimer, Miriam; Hoyle, Joseph; Elsheikh, Bakri; Arnold, David; LoRusso, Samantha
    Background: There is variability in the literature regarding the characteristics of triple seronegative myasthenia gravis (SNMG) patients. Most studies were performed before LRP4 antibodies were discovered, and characterizations of triple seronegative patients are lacking in the literature. Methods: We retrospectively investigated patients diagnosed with myasthenia gravis (MG) at Ohio State University from 2009 to 2019. Triple SNMG was defined by a history and examination that was consistent with MG and positive SFEMG, RNS or edrophonium testing, but negative serology for AChR, MUSK, and LRP4 antibodies. Results: A total of 210 AChR+, 9 MuSK+, 6 LRP4+, 9 double SNMG, and 21 triple SNMG patients were reviewed. Triple SNMG patients required significantly fewer immunosuppressive agents compared with AChR+ patients (p=0.0001) and a trend towards a less frequent history of hospitalizations, myasthenic crises and intubations compared to all antibody positive groups. Triple SNMG patients had a significantly higher frequency of ocular disease (33%) compared to AChR+ patients (13%) (p=0.0250). One triple and one double SNMG patient had thymic hyperplasia and improved after thymectomy. 11 triple SNMG patients had negative genetic testing for CMS. Conclusion: Our results further elucidate the clinical characteristics of triple SNMG, which include the predominance for ocular disease and a less severe disease course. Although likely rare, investigation for thymic pathology should be a consideration even in SNMG, and thymectomy should be considered when there is thymic pathology. We did not find alternate diagnoses in SNMG patients and thus ancillary testing should be considered in carefully selected patients for cost-effective care.
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    Charcot-Marie-Tooth: From Molecules to Therapy
    (International Journal of Molecular Sciences) Morena, Jonathan; Gupta, Anirudh; Hoyle, J Chad
    Charcot-Marie-Tooth (CMT) is the most prevalent category of inherited neuropathy. The most common inheritance pattern is autosomal dominant, though there also are X-linked and autosomal recessive subtypes. In addition to a variety of inheritance patterns, there are a myriad of genes associated with CMT, reflecting the heterogeneity of this disorder. Next generation sequencing (NGS) has expanded and simplified the diagnostic yield of genes/molecules underlying and/or associated with CMT, which is of paramount importance in providing a substrate for current and future targeted disease-modifying treatment options. Considerable research attention for disease-modifying therapy has been geared towards the most commonly encountered genetic mutations (PMP22, GJB1, MPZ, and MFN2). In this review, we highlight the clinical background, molecular understanding, and therapeutic investigations of these CMT subtypes, while also discussing therapeutic research pertinent to the remaining less common CMT subtypes.
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    Facial Onset Sensory and Motor Neuronopathy: A Case Series and Literature Review
    (RRNMF Neuromuscular Journal) Morena, Jonathan; Kamdar, Hera; Yasin, Rabia; Hoyle, J Chad; Quick, Adam; Kolb, Stephen
    Introduction: Facial Onset Sensory and Motor Neuronopathy (FOSMN) typically presents with paresthesias in the trigeminal nerve distribution and weakness that progresses rostro-caudally. Objective: To present two new cases of FOSMN, summarize the current literature, and address areas for future study. Methods: Observational data was collected from two patients with FOSMN from our institution. A literature review of FOSMN was completed using PubMed. Results: We identified 100 cases of FOSMN, including our two new cases. 93% presented with facial paresthesias. 97% had bulbar symptoms. Five had family history of ALS. Abnormal Blink reflex was most common on EMG/NCS. CSF was typically normal, but a rare severe case showed elevated protein. Mutations included: TARDBP, OPMD, D90A-SOD1, CHCHD10, VCP, and SQSTM1. Neuropathological studies showed neurodegenerative changes without inflammation. Some cases have reported transient stabilization or improvement to immunomodulatory therapy. Case Reports: A 72-year-old man presented with right-sided trigeminal paresthesias that progressed in a rostro-caudal fashion, dysphagia, and hand weakness. He died 4-5 years after symptom onset. A 69-year-old man presented with left-sided jaw paresthesias, dysphagia and dysarthria. He was trialed on IVIG for 1.5 years without improvement and died 2.6 years after symptom onset. Conclusion: FOSMN is a rare disorder with a unique clinical and electrophysiological phenotype. The pathophysiology has been associated with neurodegeneration and multiple gene mutations have correlated to FOSMN. Some reports suggest transient response to immunomodulatory therapy, though prospective studies are lacking. CSF protein elevation may be seen in severe disease. Future studies will help further elucidate the approach to diagnosis, treatment, and prognostic counseling (biomarkers).  
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    Health Consequences of Thymus Removal in Adults.
    (The New England journal of medicine, 2023-11) Morena, Jonathan; Duke Myasthenia Gravis Physicians
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    Intraneural ganglion cyst of the peroneal nerve at the lateral knee: A case report and literature review
    (RRNMF Neuromuscular Journal) Morena, Jonathan; Yang, Brian; Lee, Steve; Paul, Dustin; Leung, Dora
    Introduction: Intraneural ganglion cysts can arise from the peroneal nerve at the lateral knee secondary to synovial fluid tracking along the articular branch and transforming within the nerve into a mucinous cyst, resulting in nerve compression.   Case Report: A 17-year-old right-handed male presented with a four-month history of right foot drop. He is physically active and attributed the foot drop to a sprained ankle. EMG/NCS showed a right common peroneal neuropathy distal to the innervation of the biceps femoris short head with active denervation. MRI showed an intraneural ganglion cyst in the common peroneal nerve starting at the level of biceps femoris. On exam, he had right foot drop and sensory deficits referable to the peroneal distribution, along with a right steppage gait. He had successful decompression of the ganglion cyst, excision of the articular branch and resection of the proximal tibiofibular joint, with clinical improvement.   Conclusion: Early recognition and surgical treatment leads to better outcomes for patients when an intraneural ganglion cyst results in neurologic deficits. Physical activities and trauma, which increase stress on the knee joints, may predispose ganglion cyst formation within peroneal nerves. Fibers of the deep peroneal nerve may be preferentially affected when compared to the superficial peroneal nerve. Disconnection of the articular branch and proximal tibiofibular joint resection may decrease risk of recurrence.
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    Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease and Transverse Myelitis Probably Associated With SARS-CoV-2 mRNA Vaccines: Two Case Reports.
    (The Neurohospitalist, 2022-07) Morena, Jonathan; Gyang, Tirisham V
    Post-vaccination CNS demyelinating syndromes have been reported with a variety of vaccines including the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccines. We report a case of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) probably associated with the mRNA-1273 (by Moderna) SARS-CoV-2 mRNA vaccine, and a case of acute transverse myelitis (ATM) probably associated with the BNT162b2 (by Pfizer-BioNTech) SARS-CoV-2 mRNA vaccine. A 38-year-old man developed left blurry vision, lower extremity weakness/paresthesia, and bowel/bladder dysfunction three days after receiving the Moderna vaccine. He was diagnosed with left optic neuritis and longitudinally extensive transverse myelitis; he tested positive for the myelin oligodendrocyte glycoprotein antibody. A 39-year-old woman presented with progressive lower extremity weakness/numbness 7 days after receiving the Pfizer vaccine. She was diagnosed with ATM. Both patients improved with intravenous corticosteroids. The association between CNS demyelinating syndromes and vaccination has been reported for many years. We describe two cases of acute CNS demyelinating events probably associated with both mRNA variations of the SARS-CoV-2 vaccines. While the risk of CNS demyelinating events is non-negligible, the incidence is very low and the overall benefits of vaccination outweigh the marginal risk. However, providers should be aware of this potential neurological complication of the SARS-CoV-2 mRNA vaccines.
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    Recurrent Miller Fisher: A Case Report Along With a Literature and an EMG/NCS Review
    (The Neurohospitalist, 2021-07) Morena, Jonathan; Elsheikh, Bakri; Hoyle, J Chad
    MFS has been reported to recur in 10-12% of patients. There may be a genetic component related to HLA-DR2. Anti-GAD antibodies can be present in MFS along with anti-GQ1b. Common EMG/NCS associations consist of a predominantly axonal, sensory polyneuropathy and absent H reflexes. A 32-year-old female with a history of hypothyroidism presented to our institution twice with symptoms of diplopia, lower extremity weakness and distal paresthesias occurring a year apart. She had ophthalmoplegia, reduced reflexes, and ataxia on exam. CSF showed a borderline elevated protein of 47 and white blood cells <3. She was positive for anti-GQ1b both times. Her anti-GAD65 antibody was elevated during both admissions. EMG/NCS on her first admission revealed comparatively reduced sensory nerve action potentials (SNAPs) and a normal blink reflex. Her SNAPs improved on the second admission, however, the EMG was performed only 2 days after the onset of her symptoms, limiting some early findings that may have not matured electrophysiologically. She was treated with IVIG on both occasions with rapid recovery within 5 days. This case highlights the fact that MFS can be recurrent. It also provides further evidence that anti-GAD antibodies may be associated with MFS. Reported findings of the blink reflex in MFS are diverse and further data is needed to determine if certain findings are more predominant than others. Treatment typically consists of IVIG, though steroids may also be considered for recurrence. Prognosis is generally favorable, regardless of treatment.