Hypoglossal nerve palsy after SARS-CoV-2 vaccination - report of two cases.

BACKGROUND: SARS-CoV-2 vaccination is associated with an increased risk for Bell's palsy and some other neurological disorders assumed to be of autoimmune origin. While facial nerve palsy is frequent and usually idiopathic, hypoglossal nerve palsy is rare, and a specific cause is almost always found. We firstly report two patients who developed isolated hypoglossal nerve palsy shortly after SARS-CoV-2 vaccination. CASE PRESENTATION: Two otherwise healthy patients, a 49-year-old man and a 39-year-old woman, developed unilateral hypoglossal nerve palsy 10 and 7 days after the second SARS-CoV-2-vaccination (AstraZeneca and BioNTech/Pfizer), respectively. In both subjects, needle electromyography showed denervation and rarefication of motor units. CT, MRI, examination of blood and CSF as well as ENT exam were unremarkable. In both subjects symptoms gradually improved. CONCLUSION: Due to close temporal relationship, the absence of other etiologies, and spontaneous improvement we suspect the vaccination as the cause for hypoglossal nerve palsy in both patients. This is further supported by the rarity of isolated hypoglossal nerve palsies, especially in idiopathic cases. We suggest the addition of hypoglossal nerve palsy to the list of neurological injuries potentially caused by SARS-CoV-2 vaccination.

Advance care planning in discussion: a solution or challenge with pitfalls?

OBJECTIVES: Care planning should define care at the end of life in advance. At a later stage, when patients are no longer able to do this themselves, it should serve as a guideline for health care. The aim of this article is to give a first insight into the discussions around this social instrument. STUDY DESIGN: The study design was comparing the arguments of proponents and critics. METHODS: This is a critical discourse analysis. RESULTS: A discussion of proponents and critics shows how closely this social instrument is interwoven with the areas of biopolitics, economics and governmentality. CONCLUSIONS: Further research should address the question of what quality assurance can look like.

[Zika virus infection and the nervous system]. / Zika-Virus-Infektion und das Nervensystem.

Zika virus is an arbovirus from the family of flaviviruses, which is transmitted by the mosquito Aedes aegyptii and also by the Asian mosquito Aedes albopticus. The largest observed Zika virus epidemic is currently taking place in North and South America, in the Caribbean, southern USA and Southeast Asia. In most cases the infection is an unspecific, acute, febrile disease. Neurological manifestations consist mainly of microcephaly in newborns and Guillain-Barré syndrome but other rare manifestations have also become known in the meantime, such as meningoencephalitis and myelitis. Therefore, the Zika virus, similar to other flaviviruses, has neuropathogenic properties. In particular, the drastic increase in microcephaly cases in Brazil has induced great research activities. The virus is transmitted perinatally and can be detected in the amniotic fluid, placenta and brain tissue of the newborn. Vaccination or a causal therapy does not yet exist. The significant increase in Guillain-Barré syndrome induced by the Zika virus was observed during earlier outbreaks. In the meantime, scientifically clear connections between a Zika virus infection and these neurological manifestations have been shown. Long-term studies and animal models should be used for a better understanding of the pathomechanisms of this disease.

The efficacy of thrombolysis in lacunar stroke - evidence from the Austrian Stroke Unit Registry.

BACKGROUND AND PURPOSE: Intravenous thrombolysis (ivT) with recombinant tissue plasminogen activator is established in acute ischaemic stroke. Little is known, however, about its efficacy in different stroke subtypes. METHODS: A retrospective analysis of 128 733 patients from the Austrian Stroke Unit Registry was performed. Patients were classified as lacunar (LacS) or non-lacunar ischaemic stroke (nonLacS) by use of the clinical syndrome and technical findings. Outcome parameters were the short term improvement in the stroke unit [the difference of the National Institutes of Health Stroke Scale (NIHSS) score at admission and at discharge] and the modified Rankin Scale (mRS) score at 3 months. Patients were assigned to four groups according to thrombolysis and stroke subtype. To control for confounding, patients were matched for variables identified with impact outcome and for variables of general interest (NIHSS at admission, mRS before stroke and general risk factors). RESULTS: There were four matched groups of 401 cases each. In LacS median short term improvement was 3 [interquartile range (IQR) 2-5] NIHSS points in the thrombolysed patients and 2 (IQR 1-4) in the non-thrombolysed patients (P < 0.001). In the nonLacS groups median short term improvement was 3 (IQR 1-5) in the thrombolysed and 2 (IQR 0-4) in the non-thrombolysed patients (P < 0.001). At 3-month follow-up, ivT was significantly associated with a better functional outcome in LacS (P < 0.001) and nonLacS patients (P < 0.001). Taking magnetic resonance imaging as a requirement for stroke subtyping led to similar results. CONCLUSIONS: Patients with both lacunar and non-lacunar stroke benefitted from ivT. The degree of improvement was similar in both groups.

Subjectively perceived personality and mood changes associated with subthalamic stimulation in patients with Parkinson's disease.

BACKGROUND: Clinical and ethical implications of personality and mood changes in Parkinson's disease (PD) patients treated with subthalamic deep brain stimulation (STN-DBS) are under debate. Although subjectively perceived personality changes are often mentioned by patients and caregivers, few empirical studies concerning these changes exist. Therefore, we analysed subjectively perceived personality and mood changes in STN-DBS PD patients. METHOD: In this prospective study of the ELSA-DBS group, 27 PD patients were assessed preoperatively and 1 year after STN-DBS surgery. Two categories, personality and mood changes, were analysed with semi-structured interviews. Patients were grouped into personality change yes/no, as well as positive/negative mood change groups. Caregivers were additionally interviewed about patients' personality changes. Characteristics of each group were assessed with standard neurological and psychiatric measurements. Predictors for changes were analysed. RESULTS: Personality changes were perceived by six of 27 (22%) patients and by 10 of 23 caregivers (44%). The preoperative hypomania trait was a significant predictor for personality change perceived by patients. Of 21 patients, 12 (57%) perceived mood as positively changed. Higher apathy and anxiety ratings were found in the negative change group. CONCLUSIONS: Our results show that a high proportion of PD patients and caregivers perceived personality changes under STN-DBS, emphasizing the relevance of this topic. Mood changed in positive and negative directions. Standard measurement scales failed to adequately reflect personality or mood changes subjectively perceived by patients. A more individualized preoperative screening and preparation for patients and caregivers, as well as postoperative support, could therefore be useful.

[HIV 1-associated neurocognitive disorder: current epidemiology, pathogenesis, diagnosis and management]. / HIV-1-assoziierte neurokognitive Störung : Aktuelle Epidemiologie, Pathogenese, Diagnostik und Therapie.

By restoring the immunological function the modern antiretroviral treatment of human immunodeficiency virus (HIV-1) infection has considerably lowered the incidence of opportunistic infections. As opposed to the classical manifestations of HIV-induced immunosuppression the incidence and prevalence of HIV-associated neurocognitive disorders (HAND) has not noticeably decreased and HAND continues to be relevant in daily clinical practice. At present, HAND occurs in earlier stages of HIV infection, and the clinical course differs from that before the introduction of combination antiretroviral treatment (cART). The predominant clinical manifestation is a subcortical dementia with deficits in the domains attention, concentration and memory. Signs of central motor pathway lesions have become less frequent and less prominent. Prior to the advent of cART the cerebral dysfunction could at least partially be explained by the viral load and by virus-associated histopathological findings. In patients with at least partially successfully treated infections, this relationship no longer exists, but a plethora of poorly understood immunological and probably toxic phenomena are under discussion.This consensus paper summarizes the progress made in the last 12 years in the field of HAND and provides suggestions for the diagnostic and therapeutic management.

[Parkinson's disease: current standards in diagnostics and therapy]. / Morbus Parkinson: Aktuelle Standards in Diagnostik und Therapie.

Idiopathic Parkinson's disease is still a clinical diagnosis. However, modern imaging and nuclear techniques allow very early diagnosis and lead to higher security in the differential diagnosis between idiopathic Parkinson's disease and atypical Parkinson syndromes. At early stages of the disease, modification of disease progression and symptom control are key factors of the therapy. Continuous dopaminergic stimulation is even more important at later stages with first fluctuations. In stages where conservative medical options have been exhausted continuous pump therapies with Duodopa and apomorphine are attractive options. Deep brain stimulation in the subthalamic nucleus has turned out in the last years, especially in younger patients, to be a highly successful treatment option. Deep drain stimulation requires, however, a close preoperative work-up and individual consideration of potential effects and side effects.

Do negative CCT and CSF findings exclude a subarachnoid haemorrhage? A retrospective analysis of 220 patients with subarachnoid haemorrhage.

OBJECTIVE: Subarachnoid haemorrhage (SAH) constitutes a neurological emergency. In most cases, the diagnosis is easy to establish; however, in rare cases, verification of the diagnosis is difficult. In this retrospective analysis, we report the clinical characteristics of patients with SAH who were admitted to our neurological intensive care unit. We focus on the additional diagnostic approaches in patients with a high suspicion of SAH but failure of the 'classic' diagnostic tools. METHODS: A retrospective chart review was performed for all patients in whom SAH was diagnosed between 1996 and 2008. Two hundred and twenty patients were analysed for presenting symptoms, radiological and laboratory findings, hospital course and outcome. RESULTS: A total of 220 patients were identified (mean age 50.5 years, 127 women). In 217 patients, the diagnosis was based upon cerebral computed tomography (CCT) or lumbar puncture. In three patients, the diagnostic work-up was continued because of distinct clinical signs even though CCT and cerebrospinal fluid (CSF) were negative for SAH. In these patients, vasospasm was detected by transcranial doppler sonography (TCD) and/or diagnosis of aneurysm was confirmed by conventional angiography. CONCLUSION: Subarachnoid haemorrhage with negative CCT and CSF is a rare presentation of a severe acute neurological emergency. Further diagnostic as TCD/computed tomography (CT)-A or MR-A should be considered in all patients with typical clinical presentation for SAH but unremarkable CCT and CSF as an additional diagnostic tool. Ultimately, a conventional angiography should be performed if distinct clinical signs of SAH are presented.

[Acute headache: limitations of cerebral computed tomography and analysis of cerebrospinal fluid in the diagnosis of subarachnoid haemorrhage]. / Plötzliche und stärkste Kopfschmerzen: Wie sicher ist der kombinierte Einsatz von Computertomografie und Lumbalpunktion zum Ausschluss einer Subarachnoidalblutung?

Subarachnoid haemorrhage constitutes a neurological emergency. In most cases the diagnosis is easy to establish by cerebral computed tomography or cerebrospinal fluid tap. However, in rare cases verification of the diagnosis is more difficult and a residual uncertainty remains. We describe three patients supposed to have a subarachnoid haemorrhage without pathological findings in both cerebral computed tomography and cerebrospinal fluid. In these cases vasospasm or cerebral aneurysm were detected by means of transcranial Doppler sonography and/or conventional angiography. We comment on the special features of this rare presentation of a severe acute neurological emergency, and we discuss diagnostic work-up and differential diagnoses.

[Diagnostic work-up in Whipple's disease with cerebral involvement]. / Zerebraler Morbus Whipple: Welche Diagnostik ist Sinnvoll?

The diagnostic work-up in the case of a suspected cerebral involvement of Whipple's disease involves neuroimaging and analysis of cerebrospinal fluid (CSF) including polymerase chain reaction (PCR) assays for Tropheryma whipplei. As neurological findings may be complex and unspecific, extracerebral symptoms often lead to the suspicion of Whipple's disease. We report the cases of two patients in whom the suspected diagnosis of Whipple's disease could not be proved either by endoscopy or by the analysis of CSF. Only by means of a cerebral biopsy was the diagnosis assumed and specific therapy was initiated.

[Dementia in morbus Parkinson: reasonable diagnostics and rational therapy]. / Demenz bei Morbus Parkinson: Sinnvolle Diagnostik und rationale Therapie.

Cognitive decline is a common disorder in idiopathic Parkinson's syndrome, the risk for the development of a dementia is four- to six-fold higher for Parkinsonian patients. The cognitive profile in Parkinson's disease dementia (PDD) differs from that of Alzheimer-type dementias. The affected cognitive functions include attention, executive functions, visual-spatial functions and recall. The main differential diagnosis for PDD is the Lewy body dementia (LBD), which can be differentiated through the temporal development of motor and cognitive symptoms. Cognitive symptoms in Parkinsonian syndromes have a relevant negative impact on quality of life, on the burden for the care-givers, on the prognosis of the disease and on the possible referral to a nursing home. Dementias in Parkinsonian syndromes (PDD and LBD) need a confirmatory diagnosis at an early stage in order to initiate further therapeutic steps with, e. g., acetylcholine esterase inhibitors or, perspectively, neuropsychological training methods.

[Mistaking a long QT syndrome for epilepsy: does every seizure call for an ECG?]. / Fehldiagnose Epilepsie beim Long-QT-Syndrom: Sollte bei jedem Anfall ein EKG abgeleitet werden?

Syncope is a common and difficult differential diagnosis for epilepsy. One possible cause for a cardiac syncope is a long QT syndrome (LQTS). LQTS with torsade de pointes tachycardia can lead to lethal ventricular fibrillation and cardiac arrest. Patients with LQTS when first diagnosed as suffering from epileptic fits often experience a particularly long diagnostic delay which may even take years. In some cases, the diagnosis of LQTS is not made until the patient needs resuscitation due to a cardiac arrest. Therefore, ECG recording should be performed for every patient presenting with a seizure considered to be of epileptic origin not only at the beginning of the disease but also when fits occur in spite of antiepileptic treatment in order to prevent an incorrect diagnosis and delay in making the correct diagnosis.

[Thrombolytic therapy in conversion disorder with sensorimotor hemisyndrome]. / Lysetherapie bei Konversionsstörung mit sensomotorischem Hemisyndrom.

We here report on a 43-year-old man who was repeatedly admitted to our stroke unit with acute onset of sensorimotor hemisyndrome of acute onset. In most cases symptoms ceased shortly after admission, but twice when symptoms persisted thrombolytic therapy was applied. This case demonstrates that in emergency situations a rare differential diagnosis like conversion disorder with sensorimotor deficits may be hard to establish even if the patient presents to the same emergency unit.

Correlation of anatomy and function in medulla oblongata infarction.

BACKGROUND: A presentation of all aspects of the dorsolateral medulla oblongata syndrome is clinically very rare to find. In most cases patients present with fragmentary symptoms, e.g. ipsilateral axial lateropulsion, nystagmus, dysarthria, dysphagia or hemiataxia. However, the clinical presentation and lesion anatomy at the level of the medulla oblongata is still unsatisfactory. The aim of this study was to correlate the functional deficit with structural MRI-data. METHODS: We included thirteen patients (eight male, five female, mean age 65.5) with medulla oblongata infarction with clinically predominant ipsilateral axial lateropulsion and correlated clinical with structural deficits. RESULTS: Magnetic resonance imaging lesion mapping demonstrated ipsilateral axial lateropulsion to result from lesions of the spinocerebellar tract, the inferior cerebellar peduncle or the inferior vestibular nucleus. Nystagmus was associated with lesions of the inferior vestibular nucleus, dissociated sensory loss with the spinothalamic tract and hemiataxia with the spinocerebellar tract. CONCLUSIONS: Correlating dysfunction and lesion anatomy is a promising approach to enhance our knowledge on medulla oblongata topography.

High resolution positron emission tomography demonstrates basal ganglia dysfunction in early Parkinson's disease.

High resolution positron emission tomography (PET) with the newly developed HRRT scanner (Siemens/CTI) permits the reliable quantification of 18-Fluorodeoxyglucose (FDG) uptake as a marker of neuronal activity in small subcortical nuclei which are involved in the pathophysiology of Parkinson's disease (PD). We investigated the normalized cerebral metabolic rates of glucose (nCMRGlc) with HRRT PET in basal ganglia (BG) nuclei of 10 early-stage PD patients and in 9 healthy volunteers. PET data were co-registered to magnetic resonance images and analyzed in a three-dimensional volume-of-interest (VOI) approach. After normalization for global brain activity, PD patients showed a significantly higher nCMRGlc than controls bilaterally in the BG output nuclei (pallidum, substantia nigra) and unilateral in the caudate and putamen. The metabolic activity of the nucleus accumbens, the subthalamic nucleus, the corpus amygdaloideum and the red nucleus was normal. These first HRRT PET data in living parkinsonian humans extend previous brain imaging findings of abnormal network activity in the BG and confirm output nuclei and striatal overactivation also in early stage PD patients.

[Early development of childhood-onset schizophrenia]. / Frühentwicklung kindlicher Schizophrenien.

Childhood-onset schizophrenia (COS, age of onset between 7 and 14 years) is a rare and severe form of the disorder. The prevalence is about 1 / 50 of the rate of adult-onset schizophrenia. In COS-children emotional, cognitive and behavioural abnormalities are often seen years before illness onset. Premorbid symptoms including social withdrawal, isolation, introversion, peculiar behaviour, unmotivated temper tantrums, auto- and heteroaggressive acts, suicidal thoughts, anxiousness, paranoid ideas, represent early warning symptoms and are associated with an unfavourable outcome. About 60 % of 67 patients with COS examined by us (44 long-term-, 23 short-term-follow-up examinations) demonstrated premorbid abnormalities prior to the onset of their psychosis. We found a significant correlation between high M-PAS-scores, insidious onset, negative PANSS-Items, and early onset of age (< 12 years). High M-PAS-Scores were positively related to long duration of psychotic and residual states, and vice versa there was a negative correlation between M-PAS and a favourable outcome (long duration of recovery states). It is necessary to identify clinical states of elevated risk for psychosis as early as possible. This is difficult especially in young patients, in whom psychical peculiarities are ambiguous, and they may develop in different directions, most of them into normalization. Nevertheless, it is important to recognize risk-groups by time and to study their development carefully. Thus they could benefit from multiprofessional family-oriented early interventions.

[Prognosis after surgical treatment of spinal metastases due to lung cancer]. / Prognose nach operativem Wirbelsäuleneingriff bei Bronchialkarzinommetastasen.

BACKGROUND: Patients with spine metastases due to lung cancer suffer from a reduced lifespan. For a more precise prognosis, it is important to define parameters which influence the individual survival time. This study reviewed the mean survival time of patients who had undergone surgery because of spine metastases due to lung cancer. It should be evaluated whether the postsurgical survival time is dependent on the length of time between diagnosis and surgery and from the histological type of the tumor. MATERIAL AND METHODS: Between January 1999 and December 2003, 68 patients had undergone spine surgery because of spine metastases due to lung cancer at the department of traumatology of the St. Georg General Hospital in Hamburg, Germany. Retrospective data were collected from the hospital documentary system regarding the period between diagnosis of lung cancer and date of surgical treatment, and regarding the histological type of the tumor. The postsurgical survival times were evaluated using data from the Hamburger Cancer Index and from general practitioners. These times were analysed afterward according to the defined parameters. RESULTS: The average age was 62.6 years, and 24 female and 44 male patients were included. It was possible to evaluate the survival time of 65 patients. The average survival time of those with preoperative unknown primary manifestation (20 patients) was 88 days, in the group of patients with lung cancer histories of less than 12 months (35 patients) 141 days, and with patients with lung cancer histories of lung cancer longer than 12 months (13 patients) it was 171 days. The mean survival times after surgical treatment were 122 days for patients suffering from non-small-cell lung cancer (45 patients), 128 days for those with small-cell lung cancer (20 patients), and 247 days for patients with other histological types (three patients). DISCUSSION: The prognosis of patients after spine surgery for lung cancer metastases is poor. The indication for surgical treatment of spinal metastases due to lung cancer should be critically discussed. Especially patients with unknown primary manifestation might benefit from a conservative approach. With respect to the patients' reduced lifespan, they, their relatives, and the nursing staff should be carefully informed.

Pompe disease in Austria: clinical, genetic and epidemiological aspects.

In this study, we performed a survey of infantile and late-onset Pompe disease (IOPD and LOPD) in Austria. Paediatric and neuromuscular centres were contacted to provide a set of anonymized clinical and genetic data of patients with IOPD and LOPD. The number of patients receiving enzyme replacement therapy (ERT) was obtained from the pharmaceutical company providing alglucosidase alfa. We found 25 patients in 24 families, 4 IOPD and 21 LOPD with a resulting prevalence of 1:350,914. The most frequent clinical manifestation in LOPD was a lower limb-girdle phenotype combined with axial weakness. Three patients were clinically pauci- or asymptomatic and were diagnosed because of persistent hyperCKemia. Diagnostic delay in LOPD was 7.4 ± 9.7 years. The most common mutation was c.-32-13T > G. All IOPD and 17 symptomatic LOPD patients are receiving ERT. Standardized follow-up was only available in six LOPD patients for the 6-min walk test (6minWT) and in ten for the forced vital capacity (FVC). Mean FVC did not decline (before ERT; 63.6 ± 39.7%; last evaluation during ERT: 61.9 ± 26.9%; P = 0.5) while there was a trend to decline in the mean distance covered by the 6minWT (before ERT: 373.5 ± 117.9 m; last evaluation during ERT: 308.5 ± 120.8 m; P = 0.077). The study shows a lower prevalence of Pompe disease in Austria than in other European countries and corroborates a limb-girdle phenotype with axial weakness as the most common clinical presentation, although asymptomatic hyperCKemia may be the first indication of LOPD.

Imaging of acetylcholine esterase activity in brainstem nuclei involved in regulation of sleep and wakefulness.

Positron emission tomography with 11C-N-methyl-4-piperidyl-acetate (MP4A) was applied in eight healthy volunteers and two patients with mild Alzheimer's disease (AD) to assess acetylcholine esterase (AChE) activity in magnetic resonance imaging-identified brainstem nuclei. Uptake ratios in lateral dorsal tegmental and pedunculopontine nuclei relative to cerebellum yielded reproducible values for the AChE activity in controls and reduced values in AD, more marked in a patient with complaints of disturbed sleep. Cortical AChE activity was related to the extent of cognitive impairment which was more severe in the AD patient without sleep disturbance. This preliminary observational study demonstrates the feasibility to image and assess AChE activity in small nuclei of the brain stem. This approach may be helpful to investigate the interaction of various nuclei in the complex network regulating sleep and wakefulness in representative patient groups with documented sleep disturbance.

[Surgical complications after metastatic infiltration of the spine]. / Komplikationen bei operativer Dekompression an der tumorinfiltrierten Wirbelsäule.

INTRODUCTION: This study evaluated the intra- and post-surgical complications on tumor affected spines. Complications were analyzed according to selected patient groups so that risk factors could be determined. MATERIAL AND METHODS: Between January 1999 and December 2004, 401 patients underwent surgery because of spinal metastases in the Department of Traumatology, General Hospital St. Georg in Hamburg. Data were obtained from the hospital's documentary system. The results of this study were compared to other published studies. RESULTS: The average age of patients was 63 years (24-88) and there were 172 (42.9%) females and 229 (57.1%) males. A total of 118 (29.4%) patients suffered from 235 complications and 22 (5.5%) died. DISCUSSION: Patient's age >70 years, patients with a preoperative neurological deficit, and patients with heavily bleeding metastases are at high risk for complications. The dorsoventral/dorsolateral approach had the highest complication rate.