Kyum-Yil Kwon

A case of normal pressure hydrocephalus presenting as ventriculoperitoneal shunt-responsive dementia and choreodystonia

Normal pressure hydrocephalus (NPH) is clinically characterized by progressive gait disturbance, dementia, and urinary incontinence, although the manifestations are known to vary. Features of parkinsonism including freezing of gait have been commonly shown in patients with NPH [1, 2], while chorea or dystonia has rarely been reported [2, 3]. We report a case of an elderly female diagnosed with NPH who presented with dementia and mixed movement disorder phenomenology including chorea and dystonia. A 68-year-old woman was admitted to our hospital for evaluation of progressive gait difficulty and involuntary movements. The patient’s past medical history was notable for hypertension, diabetes mellitus, a stroke involving the right occipital lobe that occurred 3 years prior to this presentation, and osteoarthritis of bilateral knees. The year prior, she was diagnosed with unspecified dementia at an outside institution and was additionally prescribed an acetylcholinesterase inhibitor. Her family members did not know the specific details of her illness and they reported that the patient did not undergo neuroimaging for the diagnosis of dementia. Moreover, we could not obtain the previous medical records of the patient. We supposed that the patient might be considered as having Alzheimer’s disease based on a preexisting brain MRI at the time of stroke and neuropsychological evaluation. Since she had difficulty living independently because of her cognitive difficulty, she admitted to nursing hospital. Over the following 6 months, her gait slowly worsened, resulting in a wheelchair-bound state. Moreover, during this time, the patient developed involuntary body movements that gradually worsened. Initially, her family thought her gait difficulty and abnormal movements were associated with her presumed dementia. However, the patient’s cognitive impairment and movement symptoms quickly worsened, and the medical staff at the nursing hospital recommended that she should be evaluated by a movement disorders specialist. The patient’s daily medications were the following: aspirin 100 mg, amlodipine 5 mg, fimasartan 30 mg, furosemide 40 mg, metformin 750 mg, donepezil 10 mg, and vitamin B-complex with vitamin C (ascorbic acid 50 mg, cyanocobalamin 1 mcg, pyridoxine 1 mg, nicotinamide 25 mg, calcium 5 mg, riboflavin 6 mg, thiamine 6 mg), which showed no evidence of potential offending drug such as neuroleptics that would cause abnormal movements. She did not complain of any urinary issues and had no family history of neurological disorders. On neurological examination, the patient was found to have chorea in the neck, right distal upper extremity, and bilateral lower extremities, as well as dystonia, especially in her neck and left upper extremity (Video, the first part). Generalized bradykinesia without rigidity was detected in all extremities, except for the left upper extremity, which was found to be dystonic. The patient could neither stand nor walk without assistance. Her score on the Mini-Mental State Examination (MMSE) was 6 points, indicating severe cognitive impairment. Detailed laboratory tests including antiElectronic supplementary material The online version of this article (https://doi.org/10.1007/s10072-018-3451-x) contains supplementary material, which is available to authorized users.

Left Ventricular Ejection Fraction Predicts Poststroke Cardiovascular Events and Mortality in Patients without Atrial Fibrillation and Coronary Heart Disease

Background and Objectives It is controversial that decreased left ventricular function could predict poststroke outcomes. The purpose of this study is to elucidate whether left ventricular ejection fraction (LVEF) can predict cardiovascular events and mortality in acute ischemic stroke (AIS) without atrial fibrillation (AF) and coronary heart disease (CHD). Methods Transthoracic echocardiography was conducted consecutively in patients with AIS or transient ischemic attack at Soonchunhyang University Hospital between January 2008 and July 2016. The clinical data and echocardiographic LVEF of 1,465 patients were reviewed after excluding AF and CHD. Poststroke disability, major adverse cardiac events (MACE; nonfatal stroke, nonfatal myocardial infarction, and cardiovascular death) and all-cause mortality during 1 year after index stroke were prospectively captured. Cox proportional hazards regressions analysis were applied adjusting traditional risk factors and potential determinants. Results The mean follow-up time was 259.9±148.8 days with a total of 29 non-fatal strokes, 3 myocardial infarctions, 33 cardiovascular deaths, and 53 all-cause mortality. The cumulative incidence of MACE and all-cause mortality were significantly higher in the lowest LVEF (<55) group compared with the others (p=0.022 and 0.009). In prediction models, LVEF (per 10%) had hazards ratios of 0.54 (95% confidence interval [CI], 0.36–0.80, p=0.002) for MACE and 0.61 (95% CI, 0.39–0.97, p=0.037) for all-cause mortality. Conclusions LVEF could be an independent predictor of cardiovascular events and mortality after AIS in the absence of AF and CHD.

An Elderly Woman with Reversal of Clinical Presentation Mimicking Rapid-Onset Dystonia-Parkinsonism

Dear Editor, Rapid-onset dystonia-parkinsonism (RDP) (also classified as DYT12) is caused by ATP1A3 mutations,1 and usually appears during adolescence or early adulthood.2,3 Although a recent study showed that patients with the RDP phenotype were highly likely to have an ATP1A3 mutation, some cases did not.2 Herein we report an 81-year-old woman who presented with hemidystonia-parkinsonism that progressed rapidly over the course of 1 week. To our knowledge this is the first report of an elderly patient presenting RDP-mimicking features. An 81-year-old woman developed involuntary muscle contractions that began in the left fingers and progressed rapidly into the left arm, leg, and neck over the course of 1 week, and then stabilized. Her symptoms appeared after the physical stress associated with helping to hold her husband while he went to the bathroom. Her left eyeball had been removed due to an unknown etiology when she was in her 20s. Although she had severe bilateral knee osteoarthritis, she did not take any medication at the time of symptom onset. Her family—including her parents, siblings, children, and grandchildren—had no history of parkinsonism or dystonia. Two weeks after symptom onset she was admitted to our hospital due to a sustained course of left-side dystonia-parkinsonism that affected the left arm the most severely, followed by the left leg and left neck (first part of Supplementary Video 1 in the online-only Data Supplement). She complained of subjective dysarthria and dysphagia, but these conditions were not clinically significant. The patient had no history of memory impairment or psychiatric disorders including depression. A cognitive assessment using the Seoul Neuropsychological Screening Battery showed no cognitive impairment. Laboratory findings were unremarkable. Brain magnetic resonance imaging (MRI) and whole-spine MRI produced no findings to which the symptoms could be attributed (Fig. 1). Whole-exome sequencing revealed no pathogenic mutation, including in the ATP1A3 gene. Initially levodopa was prescribed at 300 mg/day, but this was discontinued due to unresponsiveness. We subsequently administered baclofen, procyclidine, and clonazepam, which resulted in a moderate improvement of hemidystonia-parkinsonism on the left side of her body (middle part of Supplementary Video 1 in the online-only Data Supplement). Freezing of gait developed after 2 weeks of treatment. The readministration of levodopa improved this gait freezing, while procyclidine was withdrawn because of memory impairment. Her dystonia-parkinsonism was considerably relieved 2 months later (latter half of Supplementary Video 1 in the online-only Data Supplement) and showed no aggravation during an 18-month follow-up. Studies indicate that clinical features of RDP with ATP1A3 mutations comprise asymmetry, rapid onset and stabilization within several weeks, predominant dystonia with a rostrocaudal gradient including prominent bulbar symptoms, no tremor, lack of pain, stressful triggers, and no or minimal improvement. Based on these symptoms we considered that our patient presented with rapidly progressing dystonia-parkinsonism, and with stabilization on Byoung June Ahn Kyum-Yil Kwon

Hand Tremor Questionnaire: A Useful Screening Tool for Differentiating Patients with Hand Tremor between Parkinson's Disease and Essential Tremor

Background and Purpose Hand tremor is one of the most frequent symptoms in movement disorders, and differential diagnoses for hand tremor include Parkinsons disease (PD) and essential tremor (ET). However, accurately differentiating between PD and ET in clinical practice remains challenging in patients presenting with hand tremor. We investigated whether a questionnaire-based survey could be useful as a screening tool in patients with hand tremor. Methods A questionnaire related to hand tremor consisting of 12 items was prospectively applied to patients with PD or ET in three movement-disorder clinics. Each question was analyzed, and a query-based scoring system was evaluated for differentiating hand tremors between PD and ET. Results This study enrolled 24 patients with PD and 25 patients with ET. Nine of the 12 questions differed significantly between PD and ET: 1 about resting tremor, 4 questions about action tremor, and 4 about asymmetry. A receiver operating characteristics curve analysis revealed that the 9-item questionnaire showed a good discrimination ability, with a sensitivity of 88% and a specificity of 84%. Conclusions The developed Hand Tremor Questionnaire might be a good screening tool for hand tremors in patients with PD and ET.

Elderly woman presenting with unusual propriospinal myoclonus triggered by drug-induced nausea and vomiting: Letters to the Editor

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Unusual case of idiopathic normal pressure hydrocephalus initially presenting as Parinaud's syndrome: Letters to the Editor

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Parkinsonian Patient with Comorbid Normal Pressure Hydrocephalus

Idiopathic normal pressure hydrocephalus (iNPH) is diagnosed not only by the clinical triad of gait disturbance, dementia, and urinary incontinence but also by the neuroradiological findings, while other neurodegenerative disorders should be excluded. Parkinsonism including gait difficulty is an early and common symptom in patients with iNPH [1]; however, co-existence of iNPH and other Parkinsonian disorders has been occasionally reported [2, 3]. This article presents an unusual case of Parkinson’s disease (PD) presenting with the clinical triad of iNPH, which was considerably improved after a ventriculoperitoneal shunt operation.

A middle-aged man with lying-down headache: an unusual presentation of sphenoid sinus mucocele

Headaches developed or aggravated at nighttime or when lying down arises conditions, such as hypnic headache, temporal arteritis, obstructive sleep apnea, migraine, cluster headache, paroxysmal hemicrania, or can be caused by neoplasm in the ventricle or brainstem, subdural hematoma, or medication [1, 2]. They can be unilateral, but in the majority of cases, they occur bilaterally. To our knowledge, sinus problems have been rarely reported to induce nocturnal or lying-down headache. In this case report, we describe the case of a middle-aged man with a mucocele in the right sphenoid sinus who presented with unilateral headache while lying down, including orbital pain. His headache resolved completely after undergoing endoscopic sinus surgery.

Bilateral thalamic and basal ganglia lesions in an old woman: unusual involvement of uremic encephalopathy

Clinical manifestation of uremic encephalopathy is variable including altered consciousness, confusion, seizure, or movement disorders such as tremor, myoclonus, dyskinesia, or Parkinsonism. Typical radiologic manifestation of uremic encephalopathy is involvement of basal ganglia, cortex or white matter, except thalamus [1, 2]. Recently, Jia et al. reported a case of uremic encephalopathy with metabolic acidosis showing isolated brainstem lesion [3]. However, to the best of our knowledge, a case of uremic encephalopathy involving thalamus in addition to basal ganglia has not been reported.

Reversible Dementia with Middle Cerebellar Peduncle Hyperintensity: 1-Year Follow-Up of HIV-Negative Neurosyphilis

Dear Editor, Patients with neurosyphilis exhibit variable clinical and neuroimaging findings. Since neurosyphilis is a treatable disease, diagnostic clues for its early detection are important. Here we report a middle-aged man who exhibited atypical dementia with bilateral middle cerebellar peduncle (MCP) hyperintensities, and was diagnosed with neurosyphilis without human immunodeficiency virus (HIV) infection. To our knowledge, this is the first report of HIVnegative neurosyphilis disclosing bilateral MCP lesions. A 49-year-old man presented with progressive cognitive impairment and gait disturbance with general weakness that initially appeared several months previously. He was right-handed and had received 6 years of education. He had been treated for diabetes mellitus and hypertension for 10 years. A bedside examination revealed mild spastic paraparesis in the lower limbs. Deep tendon reflexes were decreased, and no pathological reflexes were observed in any extremity. The findings of other neurological examinations including an ophthalmological evaluation were unremarkable. The score on the Korean version of the Mini Mental State Examination (K-MMSE) was 12 points and the Clinical Dementia Rating (CDR) was 2 points. Detailed neuropsychological tests using the Seoul Neuropsychological Screening Battery (SNSB) disclosed severe impairment in all cognitive domains except for language function (initial scores in Supplementary Table 1 in the online-only Data Supplement). Brain magnetic resonance imaging (MRI) showed mild cerebral atrophy and an isolated hyperintensity in bilateral MCPs (Fig. 1A). The patient underwent serological tests for syphilis, resulting in a positive rapid plasma reagin test with a titer of 1:2 and a positive Treponema pallidum latex agglutination test with a titer of 25.54 s/co (normal range: 0–0.99 s/co). However, the serological test for HIV was negative. A cerebrospinal fluid (CSF) examination revealed mild leukocytosis (6 cells/μL), an elevated protein level of 76.6 mg/dL (normal range: 15–45 mg/dL), and a negative venereal disease research laboratory test. Based on both serological and CSF findings, the patient was diagnosed with symptomatic neurosyphilis presenting as dementia with general paresis. Accordingly, he was treated with intravenous penicillin G potassium at 20 million units/day for 14 days. His cognitive impairment gradually improved during follow-up visits to the outpatient department, whereas his gait disturbance with general paresis was not significantly altered. The patient underwent follow-up studies for neurosyphilis after 1 year. His K-MMSE score was 28 points and the CDR was 0.5 points. The SNSB disclosed only frontal executive dysfunction (follow-up scores in Supplementary Table 1 in the online-only Data Supplement), suggesting a remarkable improvement of cognitive deficits. However, brain MRI showed that the hyperintensity was sustained in the bilateral MCPs (Fig. 1B). The clinical manifestations of neurosyphilis include dementia, general paresis, and craniJisang Park Kyum-Yil Kwon