Heidemarie Zach

The Onset of Nonmotor Symptoms in Parkinson's disease (The ONSET PD Study)

Nonmotor symptoms (NMS) in Parkinsons disease (PD) can precede onset of motor symptoms. Relationship between premotor symptoms onset and motor features is limited. Our aim is to describe the presence and perceived onset of NMS in PD as well as their possible association with motor phenotype. Presence and onset of NMS were assessed by a custom‐made questionnaire in 109 newly diagnosed untreated PD patients and 107 controls from 11 Spanish and Austrian centers. Seventeen of thirty‐one NMS were more common in patients than controls (P < 0.05). They were usually mild and frequently reported to occur at different time‐spans before motor symptoms. Anhedonia, apathy, memory complaints, and inattention occurred more frequently during the 2‐year premotor period. Those reported more frequently in the 2‐ to 10‐year premotor period were smell loss, mood disturbances, taste loss, excessive sweating, fatigue, and pain. Constipation, dream‐enacting behavior, excessive daytime sleepiness, and postprandial fullness were frequently perceived more than 10 years before motor symptoms. No correlation between NMS burden and motor severity, age, or gender was observed. NMS associated in four clusters: rapid eye movement sleep behavior disorder symptoms‐constipation, cognition‐related, mood‐related, and sensory clusters. No cluster was associated with a specific motor phenotype or severity. NMS are common in early unmedicated PD and frequently reported to occur in the premotor period. They are generally mild, but a patient subgroup showed high NMS burden mainly resulting from cognition‐related symptoms. Certain NMS when present at the time of assessment or in the premotor stage, either alone or in combination, allowed discriminating PD from controls.

The Clinical Evaluation of Parkinson's Tremor

Abstract Parkinson’s disease harbours many different tremors that differ in distribution, frequency, and context in which they occur. A good clinical tremor assessment is important for weighing up possible differential diagnoses of Parkinson’s disease, but also to measure the severity of the tremor as a basis for further tailored treatment. This can be challenging, because Parkinson’s tremor amplitude is typically very variable and context-dependent. Here, we outline how we investigate Parkinson’s tremor in the clinic. We describe a simple set of clinical tasks that can be used to constrain tremor variability (cognitive and motor co-activation, several specific limb postures). This may help to adequately characterize the tremor(s) occurring in a patient with Parkinson’s disease.

Cognitive Stress Reduces the Effect of Levodopa on Parkinson's Resting Tremor.

Resting tremor in Parkinson′s disease (PD) increases markedly during cognitive stress. Dopamine depletion in the basal ganglia is involved in the pathophysiology of resting tremor, but it is unclear whether this contribution is altered under cognitive stress. We test the hypothesis that cognitive stress modulates the levodopa effect on resting tremor.

7-Tesla MRI demonstrates absence of structural lesions in patients with vestibular paroxysmia

Vestibular parxoysmia (VP) is a rare vestibular disorder. A neurovascular cross-compression (NVCC) between the vestibulochochlear nerve and an artery seems to be responsible for short attacks of vertigo in this entity. An NVCC can be seen in up to every fourth subject. The significance of these findings is not clear, as not all subjects suffer from symptoms. The aim of the present study was to assess possible structural lesions of the vestibulocochlear nerve by means of high field magnetic resonance imaging (MRI), and whether high field MRI may help to differentiate symptomatic from asymptomatic subjects. 7 Tesla MRI was performed in six patients with VP and confirmed NVCC seen on 1.5 and 3.0 MRI. No structural abnormalities were detected in any of the patients in 7 Tesla MRI. These findings imply that high field MRI does not help to differentiate between symptomatic and asymptomatic NVCC and that the symptoms of VP are not caused by structural nerve lesions. This supports the hypothesis that the nystagmus associated with VP has to be conceived pathophysiologically as an excitatory vestibular phenomenon, being not related to vestibular hypofunction. 7 Tesla MRI outperforms conventional MRI in image resolution and may be useful in vestibular disorders.

The nature of postural tremor in Parkinson disease

Objective To disentangle the different forms of postural tremors in Parkinson disease (PD). Methods In this combined observational and intervention study, we measured resting and postural tremor characteristics in 73 patients with tremulous PD by using EMG of forearm muscles. Patients were measured both “off” medication (overnight withdrawal) and after dispersible levodopa-benserazide 200/50 mg. We performed an automated 2-step cluster analysis on 3 postural tremor characteristics: the frequency difference with resting tremor, the degree of tremor suppression after posturing, and the dopamine response. Results The cluster analysis revealed 2 distinct postural tremor phenotypes: 81% had re-emergent tremor (amplitude suppression, frequency difference with resting tremor 0.4 Hz, clear dopamine response) and 19% had pure postural tremor (no amplitude suppression, frequency difference with resting tremor 3.5 Hz, no dopamine response). This finding was manually validated (accuracy of 93%). Pure postural tremor was not associated with clinical signs of essential tremor or dystonia, and it was not influenced by weighing. Conclusion There are 2 distinct postural tremor phenotypes in PD, which have a different pathophysiology and require different treatment. Re-emergent tremor is a continuation of resting tremor during stable posturing, and it has a dopaminergic basis. Pure postural tremor is a less common type of tremor that is inherent to PD, but has a largely nondopaminergic basis.

Stress-echocardiography is underused in clinical practice: a nationwide survey in Austria

SummaryBackgroundThe wide area of application, including coronary artery disease, valvular heart disease, or pulmonary hypertension makes stress echocardiography (SE) a powerful, cost-effective imaging modality in cardiology. The role of this technique in clinical practice in Austria is unknown.MethodsA nationwide survey included all departments for cardiology and/or internal medicine in the years 2008 and 2013. By electronic questionnaire demographics, indication for the test, the numbers of examined cases per year, operators, and various applied techniques of SE were interrogated and completed by telephone interviews.ResultsData could be obtained from all 117 departments. In the year 2007 in 58 (50 %) and in 2012 57 (49 %) departments SE was available in Austrian hospitals. More than 100 SEs per year were performed by only four (7 %) units in the year 2007 and by five (8 %) in 2012. Physical exercise, dobutamine, and dipyridamole SE were available in 27 (46 %), 52 (90 %), and six (10 %) units in 2007, and in 15 (27 %), 52 (91 %), and five (9 %) units in 2012, respectively. In 2007 41 (71 %) and in 2012 26 (46 %) echo-labs administered contrast agents during SE. Transesophageal SE and 3D-echo was performed in one (2 %) and three (5 %) units in 2007, and in six (10 %) and four (7 %) echo-labs in 2012.ConclusionsThis representative survey demonstrates the underuse of SE in clinical practice in Austria. Even in established application fields performance is low, examination frequencies as recommended by the cardiology societies are fulfilled only by a minority of institutions.

7-Tesla MRI reveals no structural lesions of the vestibulocochlear nerve in patients with vestibular paroxysmia (VP)

WCN 2013 No: 2973 Topic: 36 — Other Topic 7-Tesla MRI reveals no structural lesions of the vestibulocochlear nerve in patients with vestibular paroxysmia (VP) P.S. Rommer, C. Kronnerwetter, H. Zach, S. Trattnig, G. Wiest. Neurology, Medical University of Vienna, Vienna, Austria; Radiology, Medical University of Vienna, Vienna, Austria Introduction: Vestibular paroxysmia is a rare entity causing recurrent attacks of vertigo. A neurovascular cross-compression (NVCC) is assumed to be responsible for the symptoms. In conventional MRI the contact between the nerve and the artery can be best shown on CISS-sequences. After years of symptoms a structural lesion in the nerve may be assumed, which has to date not yet been confirmed. The aim of the current study is to investigate whether a pathologic enhancement in the vestibulocochlear nerve following NVCC can be shown by means of 7-Tesla-MRI. Patients andmethods: Six patientswith chronic VPwere included in this study. In all patients the diagnosis was established according to the clinical symptoms, the finding of a NVCC in MRI (1.5 or 3 T) and the cessation of symptoms after the administration of gabapentine or carbamazepine. Results: All patients exhibited a NVCC on 7-Tesla-MRI, confirming the findings of conventional MRI. None of the patients showed an enhancement in the vestibulocochlear nerve at the site of the NVCC in T-Tesla-MRI. Discussion: In all our patients the results of the CISS sequence in 1.5 T or 3 T MRI could be confirmed by FLAIR sequences in 7 T MRI. None of the probands showed pathological enhancement or a structural lesion in the vestibulocochlear nerve. The findings imply that the symptoms of VP cannot be understood as vestibular hypofunction due to structural pathologies but rather as the result of sensory stimulation of the 8th nerve. doi:10.1016/j.jns.2013.07.2459 Abstract — WCN 2013 No: 1650 Topic: 36 — Other Topic Posterior reversible vasoconstriction syndrome in congenital disorders of glycosylation due to a defect in Conserved Oligomeric Golgi complex (COG4) WCN 2013 No: 1650 Topic: 36 — Other Topic Posterior reversible vasoconstriction syndrome in congenital disorders of glycosylation due to a defect in Conserved Oligomeric Golgi complex (COG4) S. Franca, E. Rodrigues, J. Pimenta, E. Teles. Unidade de Doencas Metabolicas do Hospital Pediatrico Integrado do Centro Hospitalar de Sao Joao, Centro Hospitalar de Sao Joao, Porto, Portugal Background: Congenital disorders of glycosylation (CDGs) are a rare group of metabolic diseases that have been described in only a few individuals, where phenotypes are continually being updated. Some of these disorders present hemorrhagic or prothrombotic features, but there is not a clear correlation with each individual subtype. Objective: The authors describe a patient with CDG that presented a posterior reversible vasoconstriction syndrome (PRES). Patients andmethods:An 11-year-oldmale, diagnosis of CDG at 2 years of age, due to a Conserved Oligomeric Golgi (COG)4 deficiency (identified p.R729W missense mutation) with psychomotor developmental delay, hyperactivity and poor language skills. Admitted with fever, anorexia and increased inflammatory biomarkers; 3 days later presented a sleep/wakefulness pattern change, severe insomnia and visual hallucinations. A slight increase in the blood pressure profile was seen and some hours later a bilateral amaurosis was installed. Electroencephalogram revealed bi-occipital epileptiform activity. CT brain scan disclosed bilateral parieto-occipital hypodense lesions, with erasing cortical sulci, suggestive of PRES, with a restricted diffusion pattern on theMRI. Transcranial ultrasoundwas negative for vasospasm. He was started on corticotherapy and levetiracetam with recovery of visual acuity. All the remaining study was normal including coagulation factors and causes of secondary hypertension. Discussion: CDGs have multisystemic manifestations and phenotypic presentation is in constant renewal. In the case described above, the difficulty of the diagnosis in a non-cooperative patient is added. Conclusion: The authors describe the first PRES described in these groups of patients, raising the question of a possible relationship between CDGs and PRES. doi:10.1016/j.jns.2013.07.2460 Abstract — WCN 2013 No: 2913 Topic: 36 — Other Topic Sonography — A helpful method in diagnosis of carpal tunnel syndrome WCN 2013 No: 2913 Topic: 36 — Other Topic Sonography — A helpful method in diagnosis of carpal tunnel syndrome M. Komenda, V. Wohlgenannt, E. Lindeck-Pozza, W. Grisold. Neurology, Kaiser Franz Josef Hospital, Vienna, Austria Carpal tunnel syndrome is the most common entrapment neuropathy of the upper extremity. Usually physical examination and nerve conduction velocity studies are used to confirm the diagnosis. In the last years ultrasound has gained wide acceptance for imaging of peripheral nerve diseases. Our objective was to evaluate the wrist-to-forearm ratio as an imaging method for the diagnosis of carpal tunnel syndrome. Patients with electrodiagnostically proven carpal tunnel syndrome underwent an ultrasonographic examination of the median nerve at the wrist and forearm. The cross-sectional area of the median nerve was measured at these points and used for calculating the wrist-to-forearm ratio. The resultswere comparedwith the values fromhealthy volunteers. The average cross-sectional area of the median nerve at the proximal carpal tunnel was 13.61 ± 3.05 mm in patients presenting with carpal tunnel syndrome and 7.77 mm ± 1.64 mm in healthy volunteers. At the forearm the average cross-sectional area of the median nerve was 6.98 ± 0.92 mm in patients and 7.34 ± 1.47 in the control group. The calculated wrist-to-forearm ratio was 1.95 ± 0.34 in patients and 1.06 ± 0.06 in healthy volunteers. We were able to prove that using a wrist-to-forearm ratio of 1.4 gives a 100% sensitivity for detecting patients with carpal tunnel syndrome. Calculating the wrist-to-forearm ratio is a valid imaging method and is from our point of view superior to measuring the cross-sectional area of the median nerve at the wrist alone. doi:10.1016/j.jns.2013.07.2461 Abstract — WCN 2013 No: 2668 Topic: 36 — Other Topic Initiation of regular physical activity promotes quality of life in healthy elderly people WCN 2013 No: 2668 Topic: 36 — Other Topic Initiation of regular physical activity promotes quality of life in healthy elderly people M. Griebe, C. Bahr, E. Ahlsdorf, A. Ebert, A. Gass, M.G. Hennerici, K. Szabo. Department of Neurology, Universitatsmedizin Mannheim, Mannheim, Germany Abstracts / Journal of the Neurological Sciences 333 (2013) e679–e727 e713