Rezzak Yilmaz

Botulinum toxin injections for the treatment of hemifacial spasm over 16 years

The aim of this study was to investigate the efficacy and side effects of botulinum toxin (BTX) in the treatment of hemifacial spasm (HFS). We also focused on the divergence between different injection techniques and commercial forms. We retrospectively evaluated 470 sessions of BTX injections administered to 68 patients with HFS. The initial time of improvement, duration and degree of improvement, and frequency and duration of adverse effects were analysed. Pretarsal and preseptal injections and Botox (Allergan, Irvine, CA, USA) and Dysport (Ipsen Biopharmaceuticals, Paris, France) brands were compared in terms of efficacy and side effects, accompanied by a review of papers which reported BTX treatment of HFS. An average of 34.5 units was used per patient. The first improvement was felt after 8 days and lasted for 14.8 weeks. Patients experienced a 73.7% improvement. In 79.7% of injections, no adverse effect was reported, in 4.9% erythema, ecchymosis, and swelling in the injection area, in 3.6% facial asymmetry, in 3.4% ptosis, in 3.2% diplopia, and in 2.3% difficulty of eye closure was detected. Patients reported 75% improvement on average after 314 sessions of pretarsal injections and 72.7% improvement after 156 sessions of preseptal injections (p=0.001). The efficacy and side effects of Botox and Dysport were similar. BTX is an effective and safe treatment option for HFS. No difference was determined between Botox and Dysport, and pretarsal injection is better than preseptal injection regarding the reported degree of improvement.

Developments in the Role of Transcranial Sonography for the Differential Diagnosis of Parkinsonism

In the last two decades transcranial sonography (TCS) has developed as a valuable, supplementary tool in the diagnosis and differential diagnosis of movement disorders. In this review, we highlight recent evidence supporting TCS as a reliable method in the differential diagnosis of parkinsonism, combining substantia nigra (SN), basal ganglia and ventricular system findings. Moreover, several studies support SN hyperechogenicity as one of most important risk factors for Parkinson’s disease (PD). The advantages of TCS include short investigation time, low cost and lack of radiation. Principal limitations are still the dependency on the bone window and operator experience. New automated algorithms may reduce the role of investigator skill in the assessment and interpretation, increasing TCS diagnostic reliability. Based on the convincing evidence available, the EFNS accredited the method of TCS a level A recommendation for supporting the diagnosis of PD and its differential diagnosis from secondary and atypical parkinsonism. An increasing number of training programmes is extending the use of this technique in clinical practice.

Application of the movement disorder society prodromal Parkinson's disease research criteria in 2 independent prospective cohorts: Application of Research Criteria For Prodromal PD

Background: The research criteria for prodromal PD of the MDS propose a new approach for the assessment of the individual probability of prodromal PD. These criteria require a testing of their reliability in different prospective cohorts.

Substantia nigra hyperechogenicity is related to decline in verbal memory in healthy elderly adults.

Deficits in cognition have been reported in Parkinsons disease (PD) already in the early and even in the pre‐motor stages. Whilst substantia nigra hyperechogenicity measured by transcranial B‐mode sonography (TCS) represents a strong PD marker and is associated with an increased risk for PD in still healthy individuals, its association with cognitive performance in prodromal PD stages is not well established.

Erratum: Structural Ultrasound of the Medial Temporal Lobe in Alzheimer’s Disease

Purpose One of the anatomical hallmarks of Alzheimer’s disease (AD) is the atrophy of the medial temporal lobe (MTL), yet cost-effective and broadly available methodological alternatives to the current imaging tools for screening of this brain area are not currently available. Materials and Methods Using structural transcranial ultrasound (TCS), we attempted to visualize and measure the MTL, and compared the results of 32 AD patients and 84 healthy controls (HC). The MTL and the surrounding space were defined in the coronal plane on TCS. A ratio of the height of the MTL/height of the choroidal fissure (M/F) was calculated in order to obtain a regional proportion. Results An insufficient temporal bone window was identified in 22 % of the AD patients and 12 % of the HCs. The results showed that the ratio of M/F was significantly smaller in the AD group on both sides (p = 0.004 right, p = 0.007 left side). Furthermore, the M/F ratio made it possible to discriminate AD patients from HCs with a sensitivity of 83 % (right)/73 % (left) and a specificity of 76 % (right)/72 % (left) which is basically comparable to results published for magnetic resonance imaging. The measurements showed substantial intra/interrater reliability (ICC:0.79/0.69). Conclusion These results suggest that utilization of structural TCS may possibly constitute a cheap and easy-to-use supplement to other techniques for the diagnosis of AD. It may be especially useful as a screening tool in the large population of individuals with cognitive decline. Further studies are needed to validate this novel method.

Sudden Onset of Oromandibular Dystonia after Cerebellar Stroke

Background We present the case of a 65-year-old female with sudden-onset involuntary mouth opening, deviation of the jaw, facial grimacing, and tongue movements that started 6 months prior to her admission. Case Report She was diagnosed with oromandibular dystonia. Differential diagnosis of oromandibular dystonia and various etiologies were investigated. Neuroimaging studies revealed a left cerebellar infarction. Discussion To our knowledge, this case is the first oromandibular dystonia presenting with cerebellar ischemic stroke. Possible roles of the cerebellum for the pathophysiology of oromandibular dystonia are discussed.

Re-emergent tremor in Parkinson’s disease: Clinical and accelerometric properties

Re-emergent tremor (RET) and the classical parkinsonian rest tremor were considered as two different phenomena of the same central tremor circuit. However, clinical and accelerometric characteristics of these tremors were not previously compared in a single study. We evaluated disease characteristics and accelerometric measurements of two tremor types in 42 patients with Parkinsons disease. Disease specific features and accelerometric measurements of peak frequency, amplitude at peak frequency and the root mean square (RMS) amplitude of two tremor types were compared. Eighteen patients had RET and the mean latency of the RET was 9.48 (±9.2)s. Groups of only rest tremor and RET did not differ significantly in age of disease onset, disease duration and severity and mean levodopa equivalent dose. Comparison of peak frequency and amplitude at peak frequency were not different between the groups, but RMS amplitude was significantly higher in the RET group (p=0.03). RMS amplitude of RET was also correlated with disease severity (r=.48, p=0.04). These results support the previous notion that rest tremor and RET are analogue, both are triggered by the same central ossilator with RET being only the suppression of the rest tremor due to arm repositioning.

Cognitive Performance Patterns in Healthy Individuals with Substantia Nigra Hyperechogenicity and Early Parkinson's Disease.

Introduction: Hyperechogenicity of the substantia nigra (SN+) is a risk marker for Parkinson’s disease (PD) which can be detected before the diagnosis. In healthy individuals, SN+ has been associated with slight deficits in specific cognitive functions, suggesting cognitive impairment as a possible pre-diagnostic marker for PD. However, the pattern of cognitive deficits associated with SN+ has not yet been compared with those present in PD. Methods: Data of 262 healthy individuals with normal echogenicity (SN-) and 48 healthy individuals with SN+ were compared with 82 early stage PD patients using the “Consortium to Establish a Registry for Alzheimer’s disease” test battery. First, the test clusters (factors) were identified using a principal component analysis (PCA). Mean group performance of cognitive tests belonging to distinct factors, according to the PCA, and single subtest performances were compared using analyses of variance. Second, the number of individuals with abnormal cognitive performances (z-score < -1.0) were compared between groups. Results: Verbal memory, semantic and executive function, and praxis were identified as components of cognitive performances. The SN+ group performed significantly worse than the SN- group in tests assessing semantic and executive function, with a non-significant decrease in verbal memory. On the subtest level, individuals of the SN+ group scored significantly lower than the SN- group on the Boston Naming Test (BNT; p = 0.008). In all subtests, the percentages of PD patients with values below the cut-off for abnormal performance were higher than in the SN- group. Moreover, more individuals from the SN+ group scored below the cut-off in the BNT (SN- = 8.4%, SN+ = 20.8%, p = 0.01) and TMT-B (SN- = 6.9%, SN+ = 16.7%, p = 0.02), compared to the SN- group. Conclusion: This study confirms poorer performance of healthy individuals with SN+ compared to SN- in specific cognitive domains. However, against the SN- group, the cognitive profile of the SN+ group was not fully consistent with the profile of early PD patients. Our data argues that cognitive impairment associated with SN+ might differ slightly from that seen in early PD. Compensational mechanisms in the early phases of neurodegeneration, and the fact that only a subgroup of SN+ will develop PD, may partly explain these differences.

Appendectomy History is not Related to Parkinson’s Disease

BACKGROUND It has been suggested that appendectomy may modify the emergence of Parkinsons disease (PD) by affecting the retrograde transport of α-synuclein (α-syn) from the gastrointestinal system. OBJECTIVE To explore the possible role of appendectomy on PD. METHODS The retrospective data of the 1625 patients (839 PD, 633 non-α-syn parkinsonism and 153 controls) were compared. Disease specific measures between PD patients with (n = 69) and without (n = 770) appendectomy were also evaluated. RESULTS The prevalence of appendectomy was not significantly lower in the PD group (8.2%) compared to the other groups (8.4% and 7.9%, p = 0.98), and the odds of having PD against other diagnoses (OR) were not significantly different in the appendectomy group (OR = 0.99, p = 0.96). No difference was determined between PD patients with and without appendectomy with respect to the age of disease onset, disease duration and severity. Appendectomy-first symptom interval was not determined to be related with PD diagnosis (hazard ratio = 1.12, p = 0.39) and did not predict disease severity in the PD group (OR = 0.99, p = 0.54). Age of appendectomy (lower or higher than 20) also did not affect future PD diagnosis (Relative Risk = 0.9, p = 0.54) or the disease severity. CONCLUSIONS The results of the study suggest no effect of appendectomy on the emergence and clinical manifestations of PD. The removal of the appendix is possibly not sufficient to suppress the exposure of the brain stem to α-syn via vagal retrograde transport. Further studies are needed to elucidate the role of appendix in PD.

Mania as a presenting clinical feature of subacute sclerosing panencephalitis

Subacute sclerosing panencephalitis (SSPE) is a rare,slowly progressive disease resulting in severe neurologicaldeficit and death. Presentation with pure psychiatricsymptoms has been rarely reported in the literature [1–3].We describe a SSPE case of 20-year-old male who pre-sented with manic episode. His mother brought him to ourhospital with abnormal repetitive movements in his handsand legs and irritability. As his mother reported, he had beena successful student until he wanted to drop out of school2 years ago. He became more irritable and angry after then.First he began to go to gym but after only a week he got a jobinarestaurant.Heworkedforlonghoursandsleptverylittle.He spent his money intemperately and bought himself newclotheseveryday.Heboughtredshoeseventhoughheneverliked this color before. He was full of energy and never felttired. Then, he quitted his job and did not come home fordays. One day, his family learned that he was in prison be-cause of larceny. His mother took him to a psychiatrist and adiagnosis of manic episode was made; treatment with olan-zapine was started. As she reported, he became a differentperson in the next 6 months. He had urinary incontinence,abnormal involuntary movements and miscalculations. Hismedical history was unremarkable with regard to diseasesand substance abuse. His family history was uninformative.In his neurological examination, he was not oriented to timeand place, and impairment was detected in all cognitivefunctions. He also had generalized myoclonia and dystonia.His blood count, renal and liver function tests, erythrocytesedimentation rate, serum levels of vitamin B12 and ceru-loplasmin were within normal limits. Antibody against glu-tamic acid decarboxylase was negative. Magnetic resonanceimaging (MRI) of the brain revealed diffuse atrophy. Elec-troencephalogram showed generalized delta waves withhigh amplitude and a periodicity at every 5 s (Fig. 1). Hiscerebrospinal fluid (CSF) was acellular, with normal pres-sure, protein and sugar levels but with elevated anti-measlesIgG antibodies. Measles IgG index, which is a specificmarker of antibody production in CSF, was 2.25 (IgG index[1.5 is associated with antibody production in CSF). A di-agnosis ofSSPE wasmade.He wastreated with isoprinosineat a dose of 100 mg/kg/d and carbamazepine at a dose of400 mg/d. Generalized myoclonia showed well response tothe treatment but generalized dystonia stayed stable. After1 year, his MRI of the brain was performed again. Hyper-intense lesions were revealed in the splenium of corpuscallosum on T2 and fluid-attenuated inversion recovery(FLAIR) sequences (Fig. 2). On follow up, he died in thesecond year of his admission to our hospital.Subacute sclerosing panencephalitis is a progressivedisease of the central nervous system caused by defectivemeasles virus. The disease begins insidiously. The firstsymptoms are usually mild intellectual deterioration andbehavioral changes with decline in school success. As thedisease advances, myoclonic jerks, ataxia, partial andgeneralized seizures, dystonia or dyskinesia and dementiamay occur eventually leading to a decorticated state anddeath [1–4].