Dilek Ince Gunal

Voice abnormalities and their relation with motor dysfunction in Parkinson's disease.

Objective –  To evaluate changes in perceptual and several acoustic parameters of voice in patients with Parkinson’s disease (PD) and to find out any relation with these parameters and motor components of Unified Parkinson’s Disease Rating Scale (UPDRS) in this patient group.

The effects of rasagiline on cognitive deficits in Parkinson's disease patients without dementia: A randomized, double-blind, placebo-controlled, multicenter study

Cognitive impairment can occur at all stages of Parkinsons disease. Rasagiline is a selective monoamine oxidase type‐B inhibitor that enhances central dopaminergic transmission. Dopamine is thought to be involved in certain cognitive processes such as working memory. We assessed the effects of rasagiline on cognitive deficits in cognitively impaired, nondemented patients with Parkinsons disease. This was a randomized, double‐blind, placebo‐controlled prospective study. Patients with Parkinsons disease receiving stable dopaminergic treatment were assigned to receive rasagiline 1 mg/day or placebo for 3 months. Patients were eligible if they had impairment in 2 of 4 cognitive domains (attention, executive functions, memory, visuospatial functions) in the screening neuropsychological tests, yet did not fulfill criteria for Parkinsons disease dementia. Fifty‐five patients were randomized; 48 patients completed the study. Patients in the rasagiline group showed significant improvement in digit span–backward compared with the placebo group (P = .04), with trends favoring rasagiline in digit span total and digit‐ordering tests. Verbal fluency total score showed a significant difference in favor of rasagiline (P = .038), with trends favoring rasagiline in semantic fluency test and Stroop spontaneous corrections. The composite cognitive domain Z scores revealed a significant difference in favor of rasagiline compared with placebo in the attentional Z score (P < .005). There were no significant differences between the 2 groups in the other cognitive tests or cognitive domain Z scores. The monoamine oxidase type‐B inhibitor rasagiline may exert beneficial effects on certain aspects of attention and executive functions in nondemented patients with Parkinsons disease with cognitive impairment.

Autonomic dysfunction in multiple sclerosis: correlation with disease-related parameters.

Cardiovascular autonomic functions were investigated in a prospective, controlled study of 22 consecutive relapsing-remitting multiple sclerosis (MS) patients and 22 healthy subjects using 5 simple noninvasive tests and sympathetic skin response testing. Tests included the heart rate response to deep breathing, valsalva maneuver and standing, blood pressure response to standing and sustained hand grip, and were graded according to the Ewing and Clark classification as early, definite or severe impairment. The relationship between autonomic dysfunction and disease-related parameters such as the expanded disability status scale (EDSS) and disease duration was studied. Ninety percent of the patients had symptoms related with autonomic dysfunction, and 45.5 % had abnormal results in cardiovascular autonomic function testing with 4 patients also having abnormal sympathetic skin responses. Statistical analysis indicated that patients with a long disease duration rather than high EDSS carried a risk of autonomic involvement in MS. Both parasympathetic and sympathetic functions were impaired and this could have been easily overlooked by a standard EDSS follow-up. In this regard, autonomic function testing seems necessary in order to detect subclinical changes in MS patients and should be considered in outcome measures.

New alternative agents in essential tremor therapy: double-blind placebo-controlled study of alprazolam and acetazolamide.

Abstract Propranolol and primidone are widely used, effective agents in essential tremor although they are not tolerated by all patients. In the present study, the effectiveness of alprazolam, a triazole analog of benzodiazapine class, and acetazolamide, a carbonic anhydrase inhibitor, were investigated as symptomatic treatments for essential tremor. We studied 22 patients with essential tremor in a double-blind, cross-over, placebo-controlled design. The patients received in random order alprazolam, acetazolamide, primidone and placebo for four weeks, each separated by a two-week washout period. The study demonstrated that alprazolam was superior to placebo and equipotent to primidone, whereas there was no statistically significant difference between acetazolamide and placebo. The mean effective daily dose of alprazolam was 0.75 mg and there was not any troublesome side effect reported by the patients on alprazolam. Alprazolam can be used as an alternative agent in elderly essential tremor patients who can not tolerate primidone or propranolol.

Leukoencephalopathy with brain stem and spinal cord involvement and high lactate : A genetically proven case with distinct MRI findings

Leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation (LBSL) is a recently described disorder with autosomal recessive mode of inheritance. Lately, mutations in the DARS2 gene, which encodes mitochondrial aspartyl-tRNA synthetase, have been found as the underlying defect. We report a 19-year-old male patient with cerebellar, pyramidal and dorsal column dysfunctions and specific magnetic resonance imaging (MRI) and characteristic magnetic resonance spectroscopy (MRS) abnormalities. The patient was compound-heterozygous for two mutations in DARS2. MRI showed selective involvement of cerebral and cerebellar white matter and superior and inferior cerebellar peduncles, without contrast enhancement. The U-fibers were spared. The sensory and the pyramidal tracts were affected over their entire length. Involvement of the intraparenchymal trajectories of the trigeminal nerves and mesencephalic trigeminal tracts was demonstrated. In the spinal cord, signal abnormalities were identified in the dorsal columns and the lateral corticospinal tracts. Proton-MRS of the frontal and cerebellar white matter showed elevated lactate, reduced N-acetylaspartate, increased myoinositol and mildly elevated choline. In LBSL, distinct MRI findings should lead to the diagnosis, which can be confirmed by the analysis of the disease gene DARS2.

A case of multiple sclerosis with cerebral venous thrombosis: the role of lumbar puncture and high-dose steroids.

Case Report A 39-year-old woman presented with vertigo, vomiting and urgency incontinence of 10 days’ duration. Her medical history disclosed a first episode of left leg weakness lasting 1 month 2 years ago. She had no other systemic antecedents or cardiovascular risk factors. On admission, she presented left pyramidal and sensory signs together with limb ataxia. Her cranial magnetic resonance imaging (MRI) demonstrated lesions highly suggestive of a demyelinating disease. A clinical diagnosis of definite relapsing-remitting MS [4] with an Expanded Disability Status Scale (EDSS) score of 2.5 was made. An LP was performed with an atraumatic needle (18 G) on the day of admission and crebrospinal fluid (CSF) examination demonstrated the presence of oligoclonal bands with no other pathology. Consequently, pulse methylprednisolone of 1,000 mg per day for 5 days was started as an acute attack treatment, followed by 60 mg of prednisolone p.o. The patient was discharged home with partial recovery (EDSS score of 2.0) and on tapering doses of prednisolone. On the 10th day after LP and while on 50 mg of prednisolone, the patient experienced right arm weakness lasting 5 min. Twelve hours following this transient episode, she developed two consecutive, simple, partial, secondarily generalized seizures. Her neurologic evaluation revealed disorientation and somnolence, right homonymous hemianopia and right-sided pyramidal signs. Her new cranial MRI conFig. 1. T2-weighted cranial MRI showing a left cortical parietal infarct as well as periventricular hyperintense lesions compatible with MS plaques. Fig. 2. Cranial MRV demonstrating occlusion of the superior sagittal sinus.

Hemichorea associated with polycythaemia vera.

Chorea is a rare complication of polycythaemia vera. Polycythaemic chorea occurs predominantly in females and usually in generalised form. We present a 66-year-old woman with acute onset hemichorea-ballism with no vascular pathology in the basal ganglia region. A clear relationship was observed between the onset of chorea and worsening of haematological parameters in the patient. After repeated phlebotomies the patients clinical status was improved. Polycythaemic chorea must be considered, especially in the elderly, as early diagnosis leads to effective treatment and prevention of complications.

Tardive dystonia associated with olanzapine therapy

Abstract A 21-year-old man with the diagnosis of paranoid schizophrenia was admitted to our clinic with cervical dystonia developing at the end of the first year of olanzapine therapy. The present case suggests that tardive dystonia in this patient is most likely associated with olanzapine administration as this is the main antipsychotic he received. Regarding the few case reports of olanzapine-associated tardive syndromes, patients taking olanzapine should be carefully screened for the appearance of tardive movements.

Reversible valproate-induced choreiform movements

Valproate is an anticonvulsive drug whose mechanism of action is based on GABAergic systems. One of the infrequent adverse effects of valproate is choreiform movements. In our study, we report a patient having head trauma history with partial and secondary generalized seizures taking 1500 mg/day valproate. During the second month of the therapy, generalized chorea was observed. Since other aetiologic causes of chorea were excluded, acutely occurring chorea in the patient was thought to be related with valproate usage because of persistence of choreiform movements for days without any fluctuation. Valproate was stopped slowly and lamotrigine was added at a dose of 400 mg/day. Within a two-month period after cessation of the valproate, choreiform movements had disappeared. We thought that the history of head trauma and another antiepileptic drug usage were the risk factors for the occurrence of valproate-induced choreiform movements.

A case of spontaneous arm levitation in progressive supranuclear palsy

Abstract Progressive supranuclear palsy is one of the parkinsonial syndromes causing atypical parkinsonism. In recent reports, other than subcortical involvement, also cortical structures have been shown to be involved in progressive supranuclear palsy patients. One of the clinical presentations of this involvement is spontaneous arm levitation which is a component of alien limb syndrome. Here we report a clinically diagnosed progressive supranuclear palsy patient with spontaneous arm levitation. Clinically spontaneous levitation of one arm without denial of ownership suggests the presence of spontaneous arm levitation. Spontaneous arm levitation can occur in the setting of progressive supranuclear palsy and it possibly demonstrates the cortical involvement in this disorder.