Isolated Unilateral Hypoglossal Nerve Palsy Caused by Internal Carotid Artery Loop.

Isolated unilateral hypoglossal nerve (HN) palsy caused by vascular compression is a rare condition. We report a case of a 42-year-old male, presenting with tongue paresis and unilateral atrophy of the tongue due to an internal carotid artery (ICA) loop. The compression of HN by ICA loop and concomitant wall irregularities of the loop segment were observed in magnetic resonance imaging and digital subtraction angiography (DSA). The patient was managed with antithrombotic without the need of any further intervention. To our best knowledge, this is the first reported case of isolated compressive neuropathy of the HN caused by loop of the ICA. Here, the clinical presentation, etiology, and management of isolated HN palsy caused by vascular lesions are discussed along with the relevant literature.

Acute thrombosis of the carotid artery associated with positional nystagmus at multiple planes.

OBJECTIVE: An acute onset central pathology without any clear neurological symptoms may mimic peripheral vestibular problem in an emergency setting. A 54-year-old man suddenly developed dizziness without any cranial nerve symptoms, paresis, cerebellar signs or sensory disturbances except upbeat positional nystagmus at multiple provoked positions which alerted for a possible acute central pathology. FINDINGS: An instantaneous magnetic resonance imaging and angiography studies further showed obstruction of the left internal carotid artery above the bifurcation. The patient's subsequent prognosis was consistent with good recovery following anti-coagulant therapy. A follow-up MRI and angiography showed resolution of thrombosis. CONCLUSION: It should be kept in mind that positional nystagmus is likely to occur in central pathologies. Differentiation between benign paroxysmal positional vertigo and central positioning nystagmus is critical.

Decreased cerebral vasomotor reactivity in patients with obstructive sleep apnea syndrome.

OBJECTIVE: In obstructive sleep apnea syndrome (OSAS), any of the activated neural, vascular, hemodynamic, metabolic, inflammatory, and thrombotic mechanisms may be related to increased cerebrovascular disease and risk of death; however, the possible pathophysiological process between obstructive sleep apnea syndrome and stroke has not been clearly explained. We hypothesize that alterations in vasomotor reactivity in patients may be responsible for their altered cerebral blood flow, and may contribute to the increased risk of ischemic stroke. METHODS: A total of 30 untreated patients with severe obstructive sleep apnea and 26 control subjects were included in the study. The mean blood flow velocity and breath holding index were measured in middle cerebral artery bilaterally in both patient and control groups by using transcranial Doppler ultrasound. We compared the values between two groups. RESULTS: The mean blood flow velocity and breath holding indexes were significantly decreased in the patient group when compared with the control group. There were no correlations between cerebral hemodynamic parameters and polysomnographic findings in patients. CONCLUSION: Our findings suggest that there was a deteriorated vasodilator response to hypercapnia in patients with OSAS. This deterioration may stem from chemoreceptors or endothelial damages that lead to vascular relaxation and vasodilatation in cerebrovascular circulation. This impaired cerebral vascular regulation may contribute to the increased risk of stroke in patients with OSAS.

Transcallosal conduction in paroxysmal kinesigenic dyskinesia.

OBJECTIVES: Detecting whether a possible disequilibrium between the excitatory and inhibitory interhemispheric interactions in paroxysmal kinesigenic dyskinesia (PKD) exists. METHODS: This study assessed measures of motor threshold, motor evoked potential latency, the cortical silent period, the ipsilateral silent period and the transcallosal conduction time (TCT) in PKD patients. Data were compared between the clinically affected hemisphere (aH) and the fellow hemisphere (fH). RESULTS: The transcallosal conduction time from the aH to the fH was 11.8 ms (range = 2.3-20.7) and 13.6 ms (range = 2.8-67.7) from the fH to the aH. The difference in TCT in the affected side was significant (p = .019). CONCLUSION: The findings demonstrated that, although inhibitory interneurons act normally and symmetrically between the motor cortices and transcallosal inhibition was normal and symmetrical between both sides, the onset of transcallosal inhibition was asymmetrical. The affected hemisphere's inhibition toward the unaffected hemisphere is faster compared to the inhibition provided by the fellow hemisphere. These results are consistent with an inhibitory deficit in the level of interhemispheric interactions. SIGNIFICANCE: This study revealed a defect in inhibition of the motor axis could be responsible in the pathological mechanisms of kinesigenic dyskinesia.

Vestibular Evoked Myogenic Potentials in a Female Population with Migraine.

The objective is to analyze the vestibular system by vestibular evoked myogenic potential (VEMP) in 30 female patients with migraine and balance problem in a controlled study. Thirty female patients with migraine and vestibular problems were enrolled in the study (2009-2012). Fifteen age-matched healthy subjects were selected as the controls. Air conduction cervical VEMP was used. Tone-burst sound stimuli of 95 dB nHL with rarefaction polarity, 5 Hz stimulus repetition rate, 1 ms rise/fall time and 2 ms plateau time were delivered at 500 Hz. 200 sweeps were averaged. Myogenic responses were amplified and band-pass filtered (800-10 Hz). The latency and the amplitude of p1 and n1 waves and interpeak amplitude and latency differences were measured. Results were given as mean and SDs. Interaural p1 and n1 amplitude greater than 30 % asymmetry was accepted as abnormal. VEMP results were compared with controls. The One-way ANOVA test was used. Statistical significance was set at P < 0.05. VEMP responses were elicited in all controls and the patients. Comparative analysis of p1 amplitude between the patients and the controls was statistically significant (P = 0.010). P1n1 interaural amplitude difference was greater than 30 % in 4 patients (13.4 %). No statistically significant difference was found when comparing latency of all wave forms between the patients and healthy controls (P > 0.05). VEMP is an useful tool to test the vestibular system in patients with migraine and balance problem at the very early period. Clinicians should always consider migraine in patients with vertigo.

[Botulinum neuro-toxin aype-A in the treatment of chronic tension type headache associated with pericranial tenderness]. / Perikranial hassasiyet ile iliskili kronik gerilim tipi bas agrisinda botulinum toksin tip A tedavisi.

OBJECTIVES: Both peripheral and central nociceptive mechanisms are responsible in chronic TTH. Analgegics are used in the acute treatment of chronic TTH and antidepressants are used in prophylactic treatment. However, further studies are needed to bring out new treatment options. The aim of our study is to investigate the effectiveness of Botulinum Neuro-toxin Type-A (BoNTA) in the treatment of chronic TTH associated with pericranial tenderness (PT). METHODS: 14 patients with chronic TTH with PT were included in the study. 50 units Botox(®) injection was applied to the pericranial muscles (5 units for each muscles bilaterally: frontal, temporal, semispinalis capitis, spenius capitis and trapezius muscles) for each patient. Severity of headache was evaluated by VAS (Visual Analogue Scale) and number of days with headache per month were recorded before treatment and 2nd and 4th months after treatment. RESULTS: Number of days with headache per month were 19.57 ± 3.25 before treatment, 15.28 ± 4.37 at the 2nd month after treatment and 15.78 ± 3.90 at the 4th month after treatment. Severity of headache was 65.71 ± 9.16 before the treatment, 50.71 ± 13.56 at the 2nd month after treatment and 54.28 ± 10.35 at the 4th month after treatment (p<0.05). Frequency and severity of headache before treatment were significantly decreased at the 2nd month after treatment and this significance continued at the 4th month after treatment (p<0.05). CONCLUSION: BoNTA treatment may be usefull in the treatment of patients with chronic TTH associated with PT.

Effect of pramipexole on cutaneous-silent-period parameters in patients with restless legs syndrome.

OBJECTIVE: The aim of this study was to investigate cutaneous-silent-period (CSP) parameters in patients with restless legs syndrome (RLS) and examine the effects of treatment on CSP which, to our knowledge, have not been investigated till date. METHODS: A total of 25 patients with RLS and 25 healthy volunteers were studied. CSP latency and duration in the upper and lower extremities were examined in the two groups. In RLS patients, the variables were examined before and after pramipexole treatment. RESULTS: Lower-extremity CSP latency was longer (106.22±11.69 ms vs. 91.67±8.53 ms; p<0.001) and CSP duration was shorter (35.50±10.91 ms vs. 49.47±6.43 ms; p<0.001) in patients, compared with controls. In the patient group, CSP durations in the upper (40.88±7.95 ms vs. 46.84±10.22 ms; p=0.006) and lower extremities (35.50±10.91 ms vs. 44.91±6.43 ms; p=0.005) were prolonged after treatment, compared with pre-treatment values. CONCLUSIONS: Small-fibre neuropathy may exist in RLS. In addition, we suggest that pramipexole may regulate cortical and spinal inhibitory mechanisms. SIGNIFICANCE: The use of CSP may aid in the diagnosis of RLS and may be used as a measure of treatment effectiveness.

The efficacy of botulinum toxin in patients with cervicogenic headache: a placebo-controlled clinical trial.

OBJECTIVE: Botulinum toxin type-A (BoNTA) has been considered a treatment option for CH. The aim of this study was to assess the effectiveness of BoNTA treatment in patients with medically resistant CH. MATERIALS AND METHODS: Forty patients with CH were included in the study. Patients in the BoNTA group (n=20) were administered 10 U of BoNTA bilaterally to the frontal muscles, 20 U to the temporal muscles, 15 U to the semispinalis capitis, 15 U to the splenius capitis, and 15 U to the trapezius muscles (total: 150 U). Patients in the placebo group (n=20) received 0.2 mL of saline administrated to the same sites. All participations were evaluated 6 and 12 weeks after treatment; side effects, the number of painful days, severity (by visual analogue scale, VAS) and frequency of pain were evaluated. RESULTS: In the BoNTA group, the severity and frequency of pain 6 and 12 weeks post treatment were significantly lower than pre-treatment levels (p<0.05). At 12 weeks post treatment, the severity and frequency of pain in the BoNTA group were lower than in the placebo group (p<0.05). CONCLUSION: The findings suggest that BoNTA was an effective treatment for CH.

Electrophysiological assessment of the autonomic nervous system in male patients with acromegaly.

BACKGROUND/AIMS: We aimed to electrophysiologically evaluate the autonomic function in acromegalic patients using sympathetic skin response (SSR) as a reflection of the sympathetic sudomotor activity and RR interval variation (RRIV) as an indicator of the cardiovagal autonomic function. METHODS: The study group consisted of 18 male acromegalics, and the control group was composed of 18 age- and sex-matched healthy subjects. Participants underwent SSR and RRIV tests. Beginning latencies and amplitudes of the median and tibial SSRs were compared among the groups. The RRIV values recorded at rest and during hyperventilation were compared among the patients and controls. RESULTS: Latencies of SSRs recorded from the palms (median) and soles (tibial) of acromegalics were significantly longer than in healthy subjects (p = 0.004, p < 0.001). The amplitude of SSR recorded from the sole (tibial) was significantly decreased (p = 0.028). The RRIVs obtained from acromegalics at rest and during hyperventilation were significantly decreased compared with those of controls (p < 0.001). The RRIVs obtained from controls prolonged significantly during hyperventilation (p < 0.001); however, in the acromegaly group, hyperventilation did not cause a significant change in RRIV (p = 0.983). CONCLUSIONS: The present study suggests that an autonomic dysfunction exists in patients with acromegaly. Dysautonomia in acromegalics may be documented by means of SSR and RRIV.

Parkinsonism caused by chronic usage of intravenous potassium permanganate.

INTRODUCTION: Chronic overexposure to manganese (Mn) may cause neuronal degeneration. Manganese intoxication is well known to induce parkinsonism. Manganese intoxication may be associated with abnormal magnetic resonance (MR) imaging (abnormal signal hyperintensity in the globus pallidus and substantia nigra on T1-weighted images). CASES: We report an unusual presentation of manganese intoxication due to administration of a combination of acetylsalicylic acid and ephedrine HCl, potassium permanganate, and vinegar melted in tap water and administered parenterally as a psychostimulant substance in 2 cases who developed symptoms resembling parkinsonism. Neurologic examination of both cases revealed disturbances of the extrapyramidal system and a characteristic "cock walk." Tremor was present in the first case, whereas it was lacking in the second one. Cranial MRI showed bilateral symmetric T1-weighted hyperintense patterns in the globus pallidus, probably because of manganese accumulation. Different levels of response to levodopa were reported in the literature; in our cases, there was no response to levodopa. CONCLUSION: Chronic overexposure to manganese may cause an atypical form of parkinsonism associated with increased T1 signal in the basal ganglia on magnetic resonance imaging.

In restless legs syndrome, during changes in vigilance, the forced EEG shifts from alpha activity to delta or high alpha may lead to the altered states of dopamine receptor function and the symptoms.

RLS cases may carry a genetic vulnerability called EEG alpha activity gate dyscontrols which appear during changes in vigilance and generally during sleep. It is triggered by forced EEG shifts either from alpha activity to delta or high alpha. Expressions of alpha activity gate dyscontrols may have a gate effect that trigger a second vulnerability-dopamine receptor specific individual sensitivity (DRSIS) and this leads to a deficiency in dopamine transmissions at diencephalospinal dopamine system (DSDS). Due to altered gene expressions in states of dopamine receptor function, DRSIS EEGs and RLS symptoms may be interpreted as follows: A. Disinhibition state is alpha activity gate dyscontrols induced inhibition of DSDS inhibitory dopamine modulations. Dopaminergic disinhibitions inhibit inhibitory interneurons of sensory and motor nuclei neurons that are involved in RLS. These sleep sensitive inhibitory interneurons possibly have GABA-ergic functions in sleep. (I) DSDS thalamic neurons' disinhibitory effects in thalamus on GABA-ergic interneurons of: (a) Intralaminar nuclei non-discriminative sensation neurons at thalamocortical premotor network leading to symptom of "a sense of urgency to move" generally referenced to legs.(b) Reticular thalamic nucleus (RTN) neurons. At polysomnography,during NREM sleep, disinhibited RTN neurons show alpha activity gate dyscontrol 1. These are recurrent subtypes of CAP in alpha band (7-12 Hz) pointing a difficulty in shifting to subtypes of CAP in low delta bands (0.25-2.5 Hz) and sleep fragmentations.(II) Supraspinal disinhibitory projections from DSDS thalamic neurons on GABA-ergic interneurons of: (a) Sensory neurons at posterior horns of spinal cord leading to dysesthesias, generally referenced to legs.(b) Medullary-reticulospinal neurons and by way of independent spinal rhythm generators on motoneurons leading to periodic limb movements in sleep.B. Activation state is an increase in symptoms. Sensory intralaminar and motor pontin nuclei neurons are in fact excitatory but are disinhibited in RLS. Due to altered gene expression, these neurons begin to perceive 'disinhibition' as reduced inhibition. Their glutamate receptors may activate deficient dopamine transmissions on RTN leading to alpha activity gate dyscontrol 2. This implies a failure in preventing shifts to frequent subtypes of CAP in high alpha and low beta bands (12-13 Hz) resulting in an increase of sensorimotor symptoms and appearance of motor restlessness, behavioral arousals and insomnia. C. Inhibition state is spontaneous relief from sensorimotor symptoms. Short or long-term synaptic plasticities of dopamine receptors towards activations initiate negative feedbacks from inhibitory interneurons. They are supported by inhibitory dopamine modulations- alertness and some awareness generally with regular high alpha EEGs, supraspinal inhibitions and a reverse movement pattern of PLMS during standing up and continuing to walk.

Cognitive dysfunction in beta-thalassemia minor.

Beta-thalassemia minor is a common, hereditary, and mostly symptomless disease. Previous studies have shown that lower hemoglobin values are associated with poorer cognitive functions. We aimed to evaluate the cognitive function in patients with beta-thalassemia minor. Thirty-two male subjects with beta-thalassemia minor and 32 sex-, age-, and education status-matched healthy subjects were enrolled in the study. Blood tests and P300 potentials were carried out. P300 potential latency in all patients was significantly longer than those in the control group (337.63+/-34.89 msec and 310.66+/-14.30 msec, respectively; t 4.046, P<0.001). The amplitudes of P300 in patient group were significantly lower than those in the control group (5.19+/-3.59 microV and 9.81+/-3.33 microV, respectively; t 5.349, P<0.001). In this study, we have found that P300 potentials are adversely affected for cognitive functions in patients with beta-thalassemia minor.

Evaluation of periodic leg movements and associated transcranial magnetic stimulation parameters in restless legs syndrome.

Restless legs syndrome (RLS), a sensorimotor disorder characterized by unpleasant sensations commonly localized in the legs, is frequently associated with periodic limb movements (PLMs) during sleep. We investigated the role of transcranial magnetic stimulation (TMS) and cortical silent period (CSP) duration as diagnostic and monitoring tools in 20 patients with primary RLS before and after 1 month of treatment and also studied 15 normal age- and gender-matched subjects. Polysomnographic assessment was undertaken and the PLM index determined in 17 of the 20 patients. We also studied the correlation between sleep efficiency index and CSP duration because of the increasing severity of the sleep disturbance and PLMs in patients with RLS. Our results demonstrate that the duration of the CSP was reduced in patients with RLS, and that dopaminergic treatment normalized this duration. There was no correlation between the PLM index and CSP duration. It may be speculated that PLMs and the CSP are due to different inhibitory mechanisms and they may be used separately as diagnostic and monitoring tools in patients with primary RLS.

Evaluation of event-related potentials in Klinefelter syndrome and idiopathic hypogonadotrophic hypogonadism.

BACKGROUND: Many studies have evaluated patients with idiopathic hypogonadothropic hypogonadotropism (IHH), but few of these studies utilize event-related potentials (P300). AIMS: To assess the cognitive functions of hypergonadotropic vs. hypogonadotropic patients. SETTINGS AND DESIGN: The study group consisted of 41 untreated IHH patients, 32 untreated Klinefelter syndrome (KS) patients, and 30 healthy control subjects. METHODS AND MATERIAL: In this study, the latency and amplitude of P300 was evaluated in 41 untreated IHH and 32 untreated KS patients and compared to healthy control subjects (average age: 30 years). Also evaluated were the patients' hormone levels. RESULTS AND CONCLUSIONS: In this study, the amplitude of P300 was found to be reduced, and the latency prolonged in IHH patients in comparison to KS patients and control subjects. In KS patients, there was no difference in latency of P300, but the amplitude was reduced when compared with the control group. Cognitive dysfunction in patients with hypogonadotropism is related to androgen hormone levels. This deficiency can affect development of the central nervous system (CNS), causing defects of CNS to varying degrees during the perinatal period. Androgen deficiency is considered to exert its effects during the period of cognitive ability development, manifest in IHH patients but not KS patients.