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.

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.

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.

Deep brain stimulation and cognitive decline in Parkinson's disease: The predictive value of electroencephalography.

Some Parkinson's disease (PD) patients treated with subthalamic nucleus deep brain stimulation (STN-DBS) develop new-onset cognitive decline. We examined whether clinical EEG recordings can be used to predict cognitive deterioration in PD patients undergoing STN-DBS. In this retrospective study, we used the Grand Total EEG (GTE)-score (short and total) to evaluate pre- and postoperative EEGs. In PD patients undergoing STN-DBS (N = 30), cognitive functioning was measured using Mini-Mental State Test and DemTect before and after surgery. Severity of motor impairment was assessed using the Unified Parkinson's Disease Rating Scale-III. Patients were classified into patients with or without cognitive decline after STN-DBS surgery. Epidemiological data, pre- and postoperative EEG recordings as well as neuropsychological and neurological data, electrode positions and the third ventricle width were compared. A logistic regression model was used to identify predictors of cognitive decline. Motor deficits significantly improved from pre- to post-surgery, while the mean GTE-scores increased significantly. Six patients developed cognitive deterioration 4-12 months postoperatively. These patients had significantly higher preoperative GTE-scores than patients without cognitive deterioration, although preoperative cognitive functioning was comparable. Electrode positions, brain atrophy and neurological data did not differ between groups. Logistic regression analysis identified the GTE-score as a significant predictor of postoperative cognitive deterioration. Data suggest that the preoperative GTE-score can be used to identify PD patients that are at high risk for developing cognitive deterioration after STN-DBS surgery even though their preoperative cognitive state was normal.

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.

VEGF transfer based on gene-modified fibroblasts using a hypoxia-induced vector to modulate neoangiogenesis in ischaemic regions of myocutaneous transplants.

The effect of a hypoxia-inducible VEGF-expressing on wound healing in an ischaemic hind leg rat model was evaluated in this study. 180 Wistar rats were assigned randomly to three groups. After ligation of the femoral artery, group 1 received pRTP801-VEGF165, group 2 untransfected fibroblasts, group 3 saline; injection was into the subcutaneous tissue, proximal and distal to the artery ligation. Biopsy specimens were obtained on days 3, 5, 7, 14 after implementation. VEGF transgene expression, vessel architecture, the amount and total area of vessel formation were investigated. Results showed a significantly higher level of VEGF protein expression in group 1 compared to group 2 (P≤0.001) throughout the investigational period. Group 1 exhibited a significant growth of CD31-positive blood vessels in the subcutaneous tissue on day 14 compared to groups 2 and 3 (P≤0.001) (group 1, 62.20±1.92; group 2, 20.60±1.67; group 3, 12.40±1.14). Alpha-SMA-positive staining also showed significant vessel growth in group 1 on day 5 (group 1, 27.00±1.87; group 2, 7.20±1.48; group 3, 10.00±1.73). These results were confirmed in the distal muscle tissue. No significant results were obtained for the proximal muscle tissue. The subcutaneous application of pRTP801-VEGF165 showed a long-lasting effect, with an increased expression of VEGF over the entire observation period. It appears that the use of fibroblasts transfected with VEGF is a promising way to increase early angiogenesis in ischaemic tissue.

[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.

Discontinuities in slow finger movements in patients with Parkinson's disease.

Slow finger movements in healthy humans are characterized by discontinuous rhythmic changes in a low frequency band about 8 Hz. These pulsatile changes in velocity are thought to present the central output of an oscillatory cerebello-thalamo-cortical network in the same frequency. Hypothesizing that patients with Parkinson's disease (PD) in the dopaminergic OFF- and ON-condition show changes in the characteristics of discontinuities compared to healthy humans, we used a 3D-ultrasound device to measure slow finger movements of 16 patients with PD and 12 age-matched controls. We provide evidence that slow finger movements of patients with PD are characterized by discontinuities in acceleration, which are significantly slower in the OFF- but not in the ON-condition compared to healthy controls. Correlation analysis between clinical motor improvement after dopaminergic medication and changes of peak frequencies and peak power of discontinuities was not significant. We conclude that the oscillatory brain network of slow finger movements is affected in PD, presenting in a lower frequency in the OFF-condition. We suggest that one factor of the modulation of this network is a dopaminergic stimulation.

[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.

123I-FP-CIT SPECT imaging of the dopaminergic state. Visual assessment of dopaminergic degeneration patterns reflects quantitative 2D operator-dependent and 3D operator-independent techniques.

UNLABELLED: 123I-N-ω-fluoropropyl-2ß-carbomethoxy-3ß-(4-iodophenyl)nortropan (123I-FP-CIT) single photon emission computed tomography (SPECT) can be evaluated by both visual assessment and quantitative analysis to assess the striatal dopamine state in vivo. The aim of our study was to investigate if visual assessment according to a predefined image grading scale reflects the results of quantitative assessment techniques. PATIENTS, METHODS: 195 patients with a clinical diagnosis of idiopathic Parkinson's disease (n = 134), atypical parkinsonian syndrome (n = 47) or essential tremor (n = 14) were examined with 123I-FP-CIT SPECT and included in this retrospective study. Results were analysed according to predefined visual patterns of dopaminergic degeneration and graded as normal (grade 5) or abnormal (grade 1-4) independently by three raters. Quantitative two-dimensional (2D) operator-dependent, manual and three-dimensional (3D) operator-independent, automated approaches were used for quantitative analysis of the specific 123I-FP-CIT tracer binding ratio (SBR) for caudate and putamen. RESULTS: Sensitivity, specificity, PPV, NPV and diagnostic accuracy of visual assessment of 123I-FP-CIT SPECT for the diagnosis of a neurodegenerative Parkinson's syndrome were 99%, 86%, 99%, 86% and 98%, respectively. Visual assessment and quantitative analysis agreed well in evaluating the degree of dopaminergic degeneration. Significant differences (p<0.001) were found between degeneration patterns. Only between the so-called eagle wing degeneration and the normal pattern no significant differences in SBR caudate and putamen were found, neither by the quantitative manual (p = 1.00; p = 0.196) nor by the quantitative automated method (p=1.0; p = 0.785). Inter-rater agreement for visual assessment was substantial for all possible pairs of the three raters (κ = 0.70 to 0.74). Strong correlations were observed between the quantitative manual and quantitative automated methods for quantification of SBR caudatum (r = 0.920, r² = 0.846, p<0.001) and SBR putamen (r = 0.908, r²=0.824, p < 0.001). CONCLUSION: Visual assessment was highly consistent with the results obtained by quantitative analysis and showed a substantial inter-rater agreement between experienced and inexperienced raters. Our findings indicate that visual assessment might be a reliable analysis approach for clinical routine.

[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.

[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.

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.

[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.

Progression of subtle motor signs in PINK1 mutation carriers with mild dopaminergic deficit.

BACKGROUND: While homozygous mutations in the PINK1 gene cause recessively inherited early-onset Parkinson disease (PD), heterozygous mutations have been suggested as a susceptibility factor. METHODS: To evaluate this hypothesis, 4 homozygous PINK1 patients with PD and 10 asymptomatic carriers of a single heterozygous mutation from a large German family (family W) were included in this study. Clinical follow-up of the heterozygous mutation carriers 3 years after the initial visit included a detailed videotaped neurologic examination using the Unified Parkinson's Disease Rating Scale III protocol and smell and color discrimination testing. At follow-up, PET with 18-fluorodopa (FDOPA) of 13 family members was obtained in order to evaluate the clinical phenotype in light of nigostriatal dopaminergic functioning. The clinical and PET data were compared to those of healthy controls. RESULTS: While there was mild worsening of clinical signs in previously affected heterozygous mutation carriers upon follow-up, 3 additional individuals had newly developed signs of possible PD. Hyposmia was found in 7 of the heterozygous mutation carriers, diminished color discrimination in 4. The homozygous mutation carriers who were all definitely affected with PD showed a severe, 60% decrease of caudate and putaminal FDOPA uptake; heterozygous offspring also had a significant 20% putaminal FDOPA uptake reduction compared to controls. CONCLUSIONS: Our findings strengthen the hypothesis that heterozygous PINK1 mutations act as a susceptibility factor to develop at least subtle Parkinson disease motor and nonmotor signs, as supported by the finding of a reduced striatal dopaminergic FDOPA uptake not only in homozygous but also, albeit to a lesser extent, in heterozygous mutation carriers.

[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.

Neurotransmitter changes in dementia with Lewy bodies and Parkinson disease dementia in vivo.

OBJECTIVE: Although Parkinson disease with dementia (PDD) and dementia with Lewy bodies (DLB) show a wide clinical and neuropathologic overlap, they are differentiated according to the order and latency of cognitive and motor symptom appearance. Whether both are distinct disease entities is an ongoing controversy. Therefore, we directly compared patients with DLB and PDD with multitracer PET. METHODS: PET with (18)fluorodopa (FDOPA), N-(11)C-methyl-4-piperidyl acetate (MP4A), and (18)fluorodeoxyglucose (FDG) was performed in 8 patients with PDD, 6 patients with DLB, and 9 patients with PD without dementia vs age-matched controls. Data were analyzed with voxel-based statistical parametric mapping and region of interest-based statistics. RESULTS: We found a reduced FDOPA uptake in the striatum and in limbic and associative prefrontal areas in all patient groups. Patients with PDD and patients with DLB showed a severe MP4A and FDG binding reduction in the neocortex with increasing signal diminution from frontal to occipital regions. Significant differences between PDD and DLB were not found in any of the radioligands used. Patients with PD without dementia had a mild cholinergic deficit and no FDG reductions vs controls. CONCLUSIONS: Patients with dementia with Lewy bodies and Parkinson disease dementia share the same dopaminergic and cholinergic deficit profile in the brain and seem to represent 2 sides of the same coin in a continuum of Lewy body diseases. Cholinergic deficits seem to be crucial for the development of dementia in addition to motor symptoms. The spatial congruence of cholinergic deficits and energy hypometabolism argues for cortical deafferentation due to the degeneration of projection fibers from the basal forebrain.