Concurrent NMDAR and GFAP Antibody Encephalitis During Pregnancy.

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an autoimmune antibody encephalitis, commonly affecting young women with comorbid ovarian teratoma. It typically presents with alteration of consciousness, psychosis, movement disorders eventually deteriorating with seizures, dysautonomia and central hypoventilation requiring critical level of care that may last weeks to months. Removal of teratoma and immunosuppressant therapy support can led to a dramatic recovery.To our knowledge, this is the first illustrated case in the literature of a pregnant woman presenting with concurrent autoimmune NMDAR and anti-glial gibrillary acidic protein(GFAP) antibody encephalitis in the setting of an ovarian teratoma. Despite the teratoma removal and receiving various forms of immunosuppressant therapy, a meaningful neurological improvement was observed following the delivery. After a prolonged hospitalisation and recovery period, the patient and her offspring made an excellent recovery highlighting the significance of early diagnosis and management.

Can IgG4-related disease present as isolated myositis?

IgG4-Related Disease (IgG4-RD)is a chronic fibroinflammatory disease typically characterized by inflammation or tumefaction of the organs involved. Skeletal muscle is not one of the typical organs involved in IgG4-RD. Isolated myositis related to IgG4-RD without common organ involvement such as lacrimal or salivary glands or retroperitoneal fibrosis is a controversial and debatable entity. Here we report a case of inflammatory myopathy in an elderly woman with several atypical clinical, lab, and histopathological findings suggestive of IgG4-related myositis. Two such case reports of IgG4-related myositis were reported in the literature review. This is a third case report of elevated IgG4 positive plasma cell infiltration in muscle with severe endomysial fibrosis and unusual myositis features (Figs. 1 and 2). This case-based review opens a possibility of a novel presentation of IgG4-RD and new pathogenesis in myositis.

Leucine-Rich Glioma-Inactivated 1 versus Contactin-Associated Protein-like 2 Antibody Neuropathic Pain: Clinical and Biological Comparisons.

Pain is a under-recognized association of leucine-rich glioma-inactivated 1 (LGI1) and contactin-associated protein-like 2 (CASPR2) antibodies. Of 147 patients with these autoantibodies, pain was experienced by 17 of 33 (52%) with CASPR2- versus 20 of 108 (19%) with LGI1 antibodies (p = 0.0005), and identified as neuropathic in 89% versus 58% of these, respectively. Typically, in both cohorts, normal nerve conduction studies and reduced intraepidermal nerve fiber densities were observed in the sampled patient subsets. In LGI1 antibody patients, pain responded to immunotherapy (p = 0.008), often rapidly, with greater residual patient-rated impairment observed in CASPR2 antibody patients (p = 0.019). Serum CASPR2 antibodies, but not LGI1 antibodies, bound in vitro to unmyelinated human sensory neurons and rodent dorsal root ganglia, suggesting pathophysiological differences that may underlie our clinical observations. ANN NEUROL 2021;90:683-690.

Isolated Third Cranial Nerve Palsy in Pituitary Apoplexy: Case Report and Systematic Review.

OBJECTIVES: To report a case of isolated third nerve palsy from pituitary apoplexy and perform a systematic literature review. MATERIALS AND METHODS: MEDLINE/EMBASE databases were searched up to September 2020. INCLUSION CRITERIA: Age≥18, isolated third nerve palsy from pituitary apoplexy. EXCLUSION CRITERIA: Age<18, presence of other neurological findings, no hemorrhage or infarction of pituitary. RESULTS: Case report: A 76-year-old woman presented with headache and right-sided ptosis. Right-eye exam revealed complete ptosis, absent pupillary constriction and accommodation, depressed and abducted eye on primary gaze, and -1 impaired depression, adduction, elevation, without other neurological findings. Brain MRI was suggestive of pituitary apoplexy. Pathology after transsphenoidal resection revealed an infarcted pituitary adenoma. Third nerve palsy resolved completely in 21 days. Systematic review: Twenty-three studies reporting 35 patients were selected from 182 abstracts. Twenty-nine (83%) had complete isolated third nerve palsy. Headache was reported in 31 (97%). Thirty-one had hemorrhage and 1 had infarction of pituitary. Cavernous sinus invasion occurred in 14 (50%). Twenty-eight were managed surgically (80%) and 7 medically (20%). Nerve palsy resolved completely in 27 (82%) and partially in 4 (11%). CONCLUSIONS: Pituitary apoplexy is an important differential diagnosis in patients with isolated third nerve palsy. Isolated third nerve palsy in apoplexy appears to have favorable prognosis.

Exercise in neuromuscular disease.

In this review, the authors present an overview of the role of exercise in neuromuscular disease (NMD). The authors demonstrate that despite the different pathologies in NMDs, exercise is beneficial, whether aerobic/endurance or strength/resistive training. The authors analyze methodological flaws of existing studies and suggest improvements for future trial protocols. Finally, we discuss specialized exercise training of specific muscles, as well as new technologies adapted from other neurologic disorders, including body-weight-supported treadmill ambulation, robotic-assisted gait training, and neuromuscular electrical stimulation.