Autoimmune encephalitis is most commonly caused by autoantibodies against N-methyl-D-aspartate (NMDA) receptors, and the malignancy most often associated with anti-NMDA receptor autoimmune encephalitis is an ovarian teratoma. Here, we describe a case of autoimmune encephalitis caused by a newly discovered cerebrospinal fluid autoantibody that has not been previously described and is not anti-NMDA receptor-mediated, which has been associated with an ovarian teratoma. It was successfully treated with high-dose corticosteroids and plasmapheresis followed by rituximab and chemotherapy (paclitaxel, ifosfamide, and cisplatin) for her teratoma.

A 58-year-old man with history of left parietal lobe ischemic stroke, multifocal lacunar infarctions with residual mild right sided weakness, ongoing tobacco abuse, and hypertension presented to the emergency department with an initial complaint of right sided throbbing headache, right neck, arm, and leg pain that started acutely 3 hours prior. Patient denied new weakness or numbness from his baseline and was ambulatory. While undergoing triage, he acutely developed right arm and leg flaccid paralysis. Upon evaluation by the stroke team he was noted to have an NIHSS of 11. CT head demonstrated chronic infarctions, and CT angiogram head and neck did not show large vessel occlusion, dissection, or focal intracranial atherosclerosis. Initial screening labs included complete blood count with differential, glucose, and complete metabolic panel all within normal range. Patient was administered rTPA and admitted to the neurologic intensive care unit. MRI brain without gadolinium did not reveal an acute infarction, but showed known prior left superior middle cerebral artery division stroke and prior lacunar infarctions. On hospital day 1 there was subjective improvement in right hemibody weakness, but also new left sided sensory deficits. Examination was notable for 4-/5 right upper extremity strength, 5-/5 right lower extremity strength, nearly at baseline from prior ischemic stroke, except for increased right hand weakness. Sensory examination demonstrated new left sided diffuse loss to pain and temperature in the upper and lower extremity with bilaterally intact vibration and proprioception.

Oromandibular dystonia (OMD) causes involuntary movements of masticatory and lingual muscles impairing eating, speaking, and swallowing. Treatment options are limited. The objective of this study was to determine the safety and efficacy of abobotulinumtoxinA (aboBoNTA) in OMD. A dose-finding study (phase 1) followed by a single session, prospective, single-blind trial (phase 2) was carried out. OMD subjects were evaluated at baseline, 6 and 12 weeks. Muscles injected were tailored to individual symptoms using EMG guidance, but the aboBoNTA dose for each muscle was pre-specified based on phase 1 results. Evaluations were Global Dystonia Rating Scale (GDS), Unified Dystonia Rating Scale (UDRS), Clinical Global Impression (CGI) improvement and severity, and quality of life (OMDQ-25). Adverse events were monitored. The lowest dosage in phase 1 resulted in adverse effects in two of three patients and thus was used in phase 2. In phase 2, adverse effects were observed in 50% of subjects including dysphagia, voice change, and soft palate weakness. Most were mild. Significant improvement was seen in quality of life (OMDQ-25), speech (BFMq21), and change in GDS, UDRS, CGI severity assessed by the unblinded investigator, but not in blinded video ratings. We conclude that aboBoNTA therapy in this study was associated with improved quality of life and was generally well tolerated in OMD, but occurrence of dysphagia dictated the importance of using low genioglossus dosing. Face to face assessment appears to be more sensitive than video assessment for change in OMD severity. Consideration of the disability in OMD places constraints on traditional placebo-control trial design. Development of novel trial designs is warranted.

To examine the safety and efficacy of AboA in OMD

The diagnosis of cervical dystonia (CD) is clinical. We describe a physical examination observation that has been noted in CD patients. There is a tendency for their shirt collars to be shifted to one side. We validated this apparently consistent finding by having blinded evaluators rating the symmetry of the shirt collars in CD and non-cervical dystonia control subjects. A high correlation was found between the physical finding which we call “shirt collar sign” and the diagnosis. “Shirt collar sign” may be a helpful sign in diagnosing CD.

This chapter explores the history of Parkinson’s disease and similar conditions. Dr James Parkinson’s initial description of this syndrome is covered along with all of the major developments in the understanding of the disease since then up to the modern era. Therapies are detailed, from early herbal remedies up to current treatment, including dopamine and levodopa, non-levodopa therapy, and surgery, and glimpses are given of potential future therapies. The conditions that are similar, the so-called “parkinsonian conditions,” are discussed as well. Synonyms: idiopathic Parkinson disease; Parkinson syndrome, Parkinson’s disease, Parkinson plus.

To analyze diffusion tensor imaging (DTI) data in the substantia nigra (SN) using a more consistent region of interest (ROI) defined by neuromelanin‐sensitive MRI in order to assess Parkinson's disease (PD) related changes in diffusion characteristics in the SN.

Background: Dentatorubropallidoluysian atrophy (DRPLA) is a rare autosomal dominant neurodegenerative disease that is associated with numerous movement disorders. Ocular problems also occur with DRPLA with reports of corneal endothelial degeneration in some patients living with the disease. We report a new visual problem associated with DRPLA, optic atrophy.Case presentation: A 47 year-old man presented complaining of progressive visual loss associated with optic atrophy on ophthalmological evaluation. He gradually developed a progressive ataxia with dystonia. Brain MRI revealed a diffuse leukoencephalopathy. Genetic analysis revealed 62 CAG repeats in one allele of the DRPLA gene and he was diagnosed with DRPLA.Conclusion: Optic atrophy should be included in the clinical spectrum of DRPLA.

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Background Valproic acid is a drug used for the treatment of a variety of psychiatric and neurological disorders. While it is well known to cause postural tremor, hyperammonemia, slowness, and sedation, it has also been described to occasionally cause a reversible form of parkinsonism. Materials and methods A series of five cases is reported. Results All patients were taking the drug for at least several months before onset of their parkinsonian symptoms. Parkinsonism was defined by the presence of bradykinesia, rigidity, postural instability, and resting tremor, but not postural or action tremor. After discontinuing their valproic acid, improvement was seen by all patients. The course of improvement took days to months after discontinuance. Two of these patients responded to dopaminergic therapy, with drug-induced dyskinesia observed in one. In another patient, valproic acid was thought to unmask underlying Parkinson's Disease; this patient benefited from levodopa as well. Conclusion Valproic acid-induced parkinsonism can look identical to idiopathic parkinsonism. In all five cases, the relationship between the valproic acid use and parkinsonism was initially unclear because of the delayed and insidious onset. Our finding of levodopa responsiveness and dyskinesia added to the diagnostic confusion. This treatment responsiveness also set it apart from neuroleptic-induced parkinsonism. In all cases improvement of symptoms occurred after discontinuation of the offending medication.