Neurol. Int., Volume 11, Issue 3 (September 2019) – 6 articles

A 69-year-old man who had been bedridden in nursing home because of a 5-year history of progressive supranuclear palsy (PSP) was admitted due to aspiration pneumonia. Besides neck dystonia in extension, he showed “alternating flexed–extended posturing”, in which the arm was flexed on one side and extended on the other. Magnetic resonance imaging of the brain revealed global cerebral atrophy that predominantly affected the cortex and midbrain. The mechanisms of complex posturing in late-stage PSP may sometimes be related to decortication and decerebration as well as dystonia, and “alternating flexed–extended posturing” might be one of the phenotypes of pathological progression in PSP. Full article

One of the best characterized autoimmune encephalitis is the Anti-Nmethyl-D-aspartate receptor (NMDAR) encephalitis, which may occur in the presence of cancer. First- and second-line immunotherapy and oncological investigations are suggested. We present here a case of an 18-year-old female who was our first patient suffering from Anti-NMDAR encephalitis more than 9 years ago. She was satisfactorily treated with intravenous immunoglobulins and high dose steroid therapy. After more than one year the patient had a relapse. First-line immunotherapy was repeated; however, a complete recovery was achieved only after plasmapheresis. Afterwards, she continued maintenance immunotherapy with steroids for two years and with Azathioprine for about five years associated to regular oncological assessment. In the last years our therapeutical approach of Anti-NMDARencephalitis has significantly changed. Nevertheless, established treatment guidelines are still missing and the role of long-term maintenance immunotherapy is largely unexplored. In addition, oncological revaluation might be indicated in selected patients. Full article

Ocular manifestations of Lyme disease (LD) remain a rare feature of the disease, but it may present a wide range of clinical presentations with different combinations. LD related optic neuritis or cranial nerve (CN) six palsy have been reported in the literature. However, this is the first case report of simultaneous involvement of CN 2 and CN 6 in a patient with LD. The diagnosis of LD can be challenging and initial laboratory tests can be a false negative. It is paramount important to repeat the diagnostic test if clinical suspicious is ongoing. With this case, we aim to increase awareness of clinicians for possible ocular manifestations of LD and its complex diagnostic process. Full article

Neuropsychiatic systematic lupus erythematosus (NPSLE) is a form of SLE involves the inflammation and/or thrombotic event in the nervous system. Patients with NPSLE are likely to have a positive antiphospholipid antibody (aPL), therefore are at higher risk of recurrent ischemic stroke. The management of NPSLE with aPLrelated stroke is rather different from the traditional ischemic stroke. One must treat it with anticoagulation and immunosuppressive therapy. The present case is a 47-yearold Taiwanese female with NPSLE and positive aPL, presented with a recurrent MCA ischemic stroke. Initial laboratory results showed significantly elevated levels of anti-ANA, anti-dsDNA, anti-cardiolipin, and decreased complement levels. Due to multiple contraindications for tPA, she was treated with antiplatelet, anticoagulation, steroid pulse therapy, and plasmapheresis during the hospitalization. Despite treatments, her stroke progressed to multi-focal lesions, involving the ACA, MCA, and basal ganglion. On follow up of her brain CT scan showed tissue edema and suspicious for subfalcine herniation. Responding to this clinical deterioration, we stopped warfarin and started mannitol. Eventually, her condition improved and was transferred to the rehabilitation program. Currently, there is no unified guideline regarding the secondary prevention of ischemic stroke in NPSLE with aPL patients. Additionally, previously reported use of steroid pulse therapy and plasmapheresis can potentially harm the patient. Clinicians must be cautious when treating such patient. Full article

In this article, we discuss the clinical approach to patients with dropped head syndrome and identify the various neuromuscular causes of dropped head syndrome including muscle, neuromuscular junction, peripheral nerve and motor neuron etiologies. We aim to increase awareness of recognition the entity of dropped head syndrome and factors that may predict response to immunomodulating therapy in dropped head syndrome. Full article

The co-existence of facioscapulohumeral muscle dystrophy (FSHD) and myasthenia gravis (MG) is very rare and few cases have been described in the literature. To increase the awareness of the health care providers, we present herein a rare case of MG in a patient with FSHD, discuss the diagnostic challenges, pre- and post-treatment findings and provide a literature review. Full article