Melis Sohtaoğlu

Long term follow-up of Parkinson's disease patients with impulse control disorders ☆

OBJECTIVE Impulse control disorders (ICDs) are mainly triggered by dopaminergic therapy in Parkinsons disease (PD). Previously, we failed to identify a relationship between the types of dopaminergic therapy and occurrence of ICDs in 33 PD patients. In this study, we aim to evaluate the outcome of ICD behaviors in the same patient group. PATIENTS AND METHODS Among 33 patients with ICDs, 22 patients were included. Demographics, dopaminergic therapy and disease severity were compared between two time points (Time 1: time of diagnosis of ICD, Time 2: last visit) and between patients who recovered from ICDs and with ongoing ICDs. Types of antipsychotic and antidepressant medication were noted. RESULTS Mean follow-up period was 43.2 months. At Time 2 mean dopamine agonist (DA) dose was significantly lower, levodopa dose and total UPDRS score was significantly higher. ICDs were dissolved in 16 patients (73%), but persisted in six (27%). In ICD(+) subgroup, DA doses in Time 1 was found significantly higher than ICD(-). However, age, PD severity, disease duration and levodopa dosage were similar. Fourteen patients were prescribed atypical antipsychotics and 13 antidepressants. In ICD(+) group, symptoms of ICDs were mainly increased libido and compulsive eating. CONCLUSIONS Although we studied a small number of patients the recovery from compulsive behaviors may be associated with the decrease in DA dosage and increase in levodopa. Furthermore, majority were given antipsychotic and/or antidepressant drugs. It is difficult to speculate which strategy could be more effective on the improvement of ICDs in such a small group. In patients who were on high doses of DA, ICDs could be persistent.

Compulsive Behaviors in Patients With Parkinson's Disease

Objective:Several impulse control disorders (ICDs) may develop in patients with Parkinsons disease (PD). We aimed to identify the frequency and phenomenology of ICDs in our PD population. Methods:Among 554 PD patients examined in a 3-year period, we identified 33 patients with ICDs. Disease duration, gender, and age-matched 65 PD patients without ICDs were selected as controls. We noted age-at-onset, duration, and severity of PD, dose and types of dopaminergic treatment, as well as presence of motor complications in both groups. Results:Of 554 patients, 33 (5.9%) had ICDs, of whom, 27 were men (81%), mean age-at onset of PD was 48 and disease duration 8 years. While all patients with ICDs were on dopamine agonist drugs (± an adjuvant), all but 2 of controls were on dopamine agonists. Punding was the most frequent behavioral problem (57%), 42% exhibited aggressive hypersexuality, 27% compulsive eating, 24% pathologic shopping, and 21% compulsive medication. Severity of PD, presence of l-Dopa-induced motor complications, l-Dopa equivalent doses of dopamine agonists administered were not statistically different between 2 groups. Conclusions:In this study performed in a tertiary clinic for movement disorders in Turkey, several ICDs occurred in a small group of PD patients, mostly in men with young-onset disease, similar to the previous reported series. However, in contrast to the Western series, the number of gamblers was quite low because gambling is illegal in our country. We did not find any association between ICDs and severity of PD as well as doses of dopaminergic agents.

Brainstem and spinal reflex studies in patients with primary progressive freezing of gait.

Our aim was to investigate the extent and pattern of involved pathways using brainstem and spinal reflexes by comparing primary progressive freezing of gait (PPFOG) progressive supranuclear palsy (PSP) with FOG. Seven patients with PPFOG and age and sex matched seven PSP patients and 16 healthy subjects were included in the study. All subjects underwent blink reflex (BR), trigemino-cervical reflex (TCR), auditory startle reflex (ASR) and long latency flexor reflex (LLFR) investigations under the same conditions. All three groups had normal BR latencies. ASR probability was lowest in the PSP group and was highest in PPFOG (p=0.005). The presence rate of TCR was lowest in PSP and it was highest in PPFOG (p=0.007 for SC and p=0.023 for SCM). The presence rate and amplitude of LLFR (R II) were decreased in the PSP group (p=0.010 and p=0.031, respectively) whereas it was in a continuous pattern in some of PPFOG patients. ASR, TCR and LLFR were all inhibited in PSP and we suggest that suppression of all three reflexes is probably related to degeneration of brainstem reticular formation and basal ganglia connections. However, interestingly, in PPFOG, excitabilities of ASR and TCR circuits are increased suggesting loss of pathways mediating suprasegmental control.

Patient with late-onset Wilson's disease: deterioration with penicillamine.

We report on a 77-year-old woman whose symptoms and laboratory findings suggested Wilson’s disease (WD) and markedly deteriorated after initiation of penicillamine therapy. When she was 67, she had developed a mild head tremor and a slight forgetfulness. At the age of 75, she developed slurred speech and tremor in both hands followed by postural instability, which caused falls within the last 6 months. Her parents had a remote consanguinity. Neurological examination revealed head titubation, cerebellar dysarthria, high-amplitude kinetic tremor in arms, dysmetria, dysdiadochokinesia, symmetrical bradykinesia, prominent postural instability, and mild cognitive impairment. MRI of the brain (October 2005) showed bilateral hyperintense lesions in the basal ganglia on T2-weighted images and a coincidental subdural hematoma on the left frontoparietal region related to a recent fall (Fig. 1A). Kaiser–Fleischer rings were present bilaterally. The 24-hour urinary copper was 231 g (normal, 0 –100) and free copper was 45 g/dL. The serum levels of copper and ceruloplasmin were normal (105 and 20 g/dL, respectively). The liver biopsy specimen revealed increased copper content on dry weight (281.1 g/g; normal, 30). Penicillamine was started at a low dose and escalated to 900 mg together with zinc 150 mg t.i.d. Two weeks later, her symptoms worsened markedly so that she became bedridden with severe Parkinsonism and a jaw-closing dystonia. The 24-hour urine copper was 457.6 g, and 1 month later 1,500 g. Although penicillamine was stopped and zinc monotherapy was continued, the clinical picture got worse. In the control MRI (March 2006), the previous lesions were more prominent (Fig. 1B) and in the last MRI (June 2006) there was an additional white matter involvement (Fig. 1C). On reexamination in June, a severe startle response was observed in addition to the previous symptoms. A few days later, she deceased due to a cardiopulmonary arrest. This case is instructive because of the late presentation of WD and the worsening effect of penicillamine. To our knowledge, she is the oldest-onset neurological WD patient reported in English literature. WD has been very seldom reported in the 6th decade.1–3 Up to now, the oldest WD patient was a 65-year-old man who developed liver failure without neurological involvement.4 A 60-year-old patient with the diagnosis of asymptomatic WD was also reported.5 To verify the diagnosis of WD on genetic analysis is difficult, because there are over 200 identified different mutations of the ATP7B gene. According to Diagnosis and Phenotypic Classification of WD,6 our patient scored 8 points, which stood for “the diagnosis of WD is highly likely.” Classically, it has been known that penicillamine is beneficial for WD patients.7 However, Brewer and colleagues8,9 have argued that neurologically presenting WD patients should never be treated with penicillamine, since symptoms may worsen within a few weeks in 52% of patients, and in 50% of whom this deterioration may become permanent and repeated MRIs may show new and severe white matter lesions, as we have observed with our patient. Because of all these concerns, Brewer and colleague9,10 have recommended for initial therapy safer drugs such as zinc, as well as tetrathiomolybdate, which is currently under investigation. Trientine has not been proposed because of its initial worsening effect in about 20% of WD patients. We conclude that the diagnosis of WD should be considered, though rarely seen, in patients with atypical neurological abnormalities, especially movement disorders, even though age at onset is older than 65 years. Moreover, it may be better to avoid penicillamine as an initial therapy, especially in elderly patients with neurological WD.

Auditory evoked blink reflex in peripheral facial paresis.

Purpose: The auditory blink reflex (ABR) is a teleceptive reflex consisting of an early brief muscle contraction of the orbicularis oculi in response to sound stimuli. Constriction of the orbicularis oculi in response to auditory stimulation is accepted as a part of the startle reaction. The blink reflex and ABR might share a final common pathway, consisting of facial nerve nuclei and the facial nerve and may have common premotor neurons. Methods: In this study, the authors evaluated the value of the ABR in patients with peripheral facial palsy (PFP), cross-checking the results with commonly used blink reflex changes. Results: In total, 83 subjects with PFP and 34 age-matched healthy volunteers were included. Auditory blink reflex was elicited in all control subjects and in 36 PFP cases on the paralytic sides (43.3%), whereas it was asymmetric in 30.1% of the patients. Auditory blink reflex positivity was significantly lower in PFP cases with increasing severity. Blink reflex results were largely correlated with ABR positivity. Conclusions: Auditory blink reflex is a useful readily elicited and sensitive test in PFP cases, providing parallel results to blink reflex and being affected by disease severity.

Emotional and behavioral influence of headache in Pediatric rheumatic diseases

• Headache can be the expression of psychological difficulties, such as anxiety and depression.

The comparison between the results and indications of electromyographic examination in childhood

Introduction: Electromyography (EMG) is one of the methods which is used in the diagnosis and prediction of prognosis of neuromuscular disorders in all age groups. Here, we aimed to determine indications and diagnosis of EMG in pediatric age group. Patients and method: All records of patients who admitted to EMG laboratory between January 2012 and December 2013, underwent nerve conduction studies and needle EMH and/or repetitive nerve stimulation tests and were under 18 years-old were retrospectively analyzed and data regarding age at admission, gender, preliminary diagnosis/ EMG indications, referral center and EMG diagnosis were recorded. Results: In the study period, 364 admissions at pediatric age were detected. Age ranges were between 3 months and 18 years (mean age: 9.9±5.7 years), 208 patients (57.1%) were male. Most frequent indications were polyneuropathy (136, 39.3%), myopathy (64, 18.5%) and mononeuropathy (35, 10.1%). EMG diagnoses were normal (235, 64.6%), myopathy (27, 7.4%), cranial neuropathy (24, 6.6%), polyneuropathy (22, 6.0%), mononeuropathy (17, 4.7%) and plexopathy (13, 3.6%). Conclusions: Most of the admissions resulted in normal findings. In pediatric age group, definitive diagnosis cannot be based on solely electrophysiological investigations. Personal and family history, neurological and physical examination findings as well as laboratory and electrophysiological findings should be evaluated altogether. Knowledge of specific syndromes and sometimes repetitive electrophysiological investigations are required.

Mentalis muscle related reflexes

The mentalis muscle (MM) arises from the incisive fossa of the mandible, raises and protrudes the lower lip. Here, we aim to characterize responses obtained from MM by supraorbital and median electrical as well as auditory stimuli in a group of 16 healthy volunteers who did not have clinical palmomental reflex. Reflex activities were recorded from the MM and orbicularis oculi (O.oc) after supraorbital and median electrical as well as auditory stimuli. Response rates over MM were consistent after each stimulus, however, mean latencies of MM response were longer than O.oc responses by all stimulation modalities. Shapes and amplitudes of responses from O.oc and MM were similar. Based on our findings, we may say that MM motoneurons have connections with trigeminal, vestibulocochlear and lemniscal pathways similar to other facial muscles and electrophysiological recording of MM responses after electrical and auditory stimulation is possible in healthy subjects.

Somatosensory and auditory startle reflex in patients with stroke and spinal cord injury

Background Somatosensory startle reflex (SSSR) was recently studied in healthy subjects. Following corticospinal tract lesions caused by stroke or spinal cord injury (SCI), auditory startle reflex (ASR) has been reported to enhance due to reorganization of circuits rostral and caudal to the lesion. To further understand changes in SSSR and ASR, we investigated both responses in patients with spinal cord injury (SCI) and stroke. Methods We examined characteristics of ASR and SSSR in 14 SCI and 40 stroke patients (16 brainstem and 24 cerebral hemispheric infarctions) and 39 age and gender matched healthy subjects. ASR was obtained after eight auditory stimuli and SSSR was elicited after median nerve stimulation at the wrist. Surface electromyographic recordings were obtained from orbicularis oculi (O.oc), sternocleidomastoid (SCM), biceps brachii (BB) and abductor pollicis brevis (APB) muscles. Results Total ASR probabilities at distal muscles were significantly higher in patients with SCI and in stroke patients especially with brainstem infarctions. Similarly SSSR rates were increased in both patient groups compared to controls (for APB p p Conclusions In conclusion, we have found that SSR and ASR were enhanced in stroke and SCI and this enhancement was more prominent in distal muscles. Secondly, the properties of ASr and SSSR differed according to the lesion site.

13. Trigemino-cervical reflex: Clinical and neuroradiological links

muscles expected to react and decreased in others such as, for instance, the homonymous muscles of the contralateral side. The contrast between the increase and decrease of excitability in homonymous muscles is likely much more marked in SRT than in CRT. We reasoned that, if inhibition is not fully accomplished, some remaining activity would be seen in the muscles not to be responding in a StartReact paradigm using SRT and CRT. Subjects and methods: In 11 volunteers, we studied the EMG and movement occurring in the forearm muscles of the side contralateral to the one requested to react in SRT and CRT. In 25% of the trials, the IS was accompanied by a SAS. Results: As expected, reaction time was shorter for trials with SAS than for trials without SAS. This was the case for both, SRT and CRT although the percentage shortening was significantly less for CRT. In no-SAS trials, contralateral activity was absent in SRT, 4% of trials in ART and 18% of trials in CRT. In SAS trials, contralateral activity was seen in 52% of the trials in SRT, in 61% in ART and in 79% in CRT. Conclusions: The difference between SAS and no-SAS trials in contralateral EMG activity may be explained by the fact that SAS causes precipitated execution preventing the inhibitory action to be fully implemented. Contralateral hand activity could also be a startle-related response but the fact that it is seen in a significant number of trials with no SAS and the fact that it takes a distribution of the triphasic pattern in a few cases makes it unlikely.