Ahmet Acarer

Primary medullary haemorrhage with intractable hiccup

Sirs: Primary medullary haemorrhage is uncommon, the clinical presentation is variable and generally characterized by a combination of unilateral pontine and medullary signs [2–5, 7, 9]. Most patients with medullary haemorrhage present with a characteristic syndrome of sudden onset of headache and vertigo together with neurological signs that correspond to medullary involvement. Hiccup is a repeated involuntary, spasmodic contraction of the diaphragm accompanied by a sudden closure of the glottis, which is reported commonly in patients with brain stem disease such as ischaemic stroke, dolichoectatic basilar artery, tumour, encephalitis and multiple sclerosis [1, 6, 8, 10]. We observed an unusual case with only intractable hiccup among the three consecutive patients with medullary haemorrhage admitted to our centre from 1 January 1994 to 31 September 31 1997. A previously healthy 44-year-old man with heavy tobacco and alcohol use was admitted to the internal medicine department of a regional hospital because of severe hiccup refractory to all medicaments and a sensation of tingling below the right knee. Clinical and gastroesophageal examinations including endoscopy, pH monitoring and manometry were all within normal limits. Ten days after the onset, the patient was referred to our clinic for neurological examination. We observed hiccup without neurological abnormalities. He had no motor, superficial and deep sensory, or lower cranial nerve abnormalities. Hiccup was persistent and severe; the patient could not eat, drink or talk. Routine haematologic and cardiological screenings were normal. Cervical medulla and brain stem were systematically explored using sagittal and axial T1and T2weighted images. Magnetic resonance imaging (MRI) with T1and T2-weighted images revealed a haemorrhage in the right medial medullary region with mild dorsal extension (Figs. 1–3). Magnetic resonance (MR) angiography and Doppler examinations revealed no abnormality. Chlorpromazine was given and arrested the hiccup 5 days after admission to our centre. At the follow-up 2 months after the stroke, he had no complaint and no hiccup. Spontaneous primary medullary haemorrhage has rarely been reported inWestern literature [1–5]. Since the case of Kempe (1964) [7], treated and diagnosed surgically, 16 patients with pure medullary haemorrhage have been found by autopsy or by computed tomography (CT) or MRI [3]. The most common documented causes of medullary haemorrhage have been vascular malformations, including arteriovenous malformation and cavernous angioma [3]. We did not observe any vascular malformation on MR angiography or LETTER TO THE EDITORS J Neurol (1998) 245 :620–622

Diagnostic value of “dysphagia limit” for neurogenic dysphagia: 17 years of experience in 1278 adults

OBJECTIVE Neurogenic dysphagia (ND) is a prevalent condition that accounts for significant mortality and morbidity worldwide. Screening and follow-up are critical for early diagnosis and management which can mitigate its complications and be cost-saving. The aims of this study are to provide a comprehensive investigation of the dysphagia limit (DL) in a large diverse cohort and to provide a longitudinal assessment of dysphagia in a subset of subjects. METHODS We developed a quantitative and noninvasive method for objective assessment of dysphagia by using laryngeal sensor and submental electromyography. DL is the volume at which second or more swallows become necessary to swallow the whole amount of bolus. This study represents 17 years experience with the DL approach in assessing ND in a cohort of 1278 adult subjects consisting of 292 healthy controls, 784 patients with dysphagia, and 202 patients without dysphagia. A total of 192 of all patients were also reevaluated longitudinally over a period of 1-19 months. RESULTS DL has 92% sensitivity, 91% specificity, 94% positive predictive value, and 88% negative predictive value with an accuracy of 0.92. Patients with ALS, stroke, and movement disorders have the highest sensitivity (85-97%) and positive predictive value (90-99%). The clinical severity of dysphagia has significant negative correlation with DL (r=-0.67, p<0.0001). CONCLUSIONS We propose the DL as a reliable, quick, noninvasive, quantitative test to detect and follow both clinical and subclinical dysphagia and it can be performed in an EMG laboratory. SIGNIFICANCE Our study provides specific quantitative features of DL test that can be readily utilized by the neurologic community and nominates DL as an objective and robust method to evaluate dysphagia in a wide range of neurologic conditions.

Is customized vestibular rehabilitation effective in patients with Parkinson's?

BACKGROUND Patients with Parkinsons disease complain about restrictions in their daily life activities and impairment in their mobility and balance. OBJECTIVE The aim of this study was to compare the effects of vestibular rehabilitation on functioning, quality of life, balance, and postural stability in patients with Parkinsons disease. METHODS The patients with Parkinsons disease divided into a rehabilitation group (Group 1, n: 29) and a control group(Group 2, n: 11). All patients were evaluated before and after eight weeks of customized vestibular rehabilitation for motor score (Unified Parkinsons Disease Rating Scale); quality of life (Parkinsons Disease Questionnaire-39); balance (Activities-Specific Balance Confidence Scale [ABC], Timed Up and Go Test, Dynamic Gait Index [DGI], and Berg Balance Scale [BBS]); and postural stability (Modified Clinical Test for Sensory Interaction on Balance [mCTSIB]. RESULTS There were significant differences in the pre- and post-exercise ABC, BBS, and DGI scores in Group 1 (p <  0.05). A statistically significant impairment was observed in mCTSIB (firm and foam eyes closed [EC]) in the control group (p <  0.05). There were no significant intergroup differences in any of the parameters evaluated (p >  0.05). CONCLUSION In this study, vestibular rehabilitation was found to be effective for improving balance in patients with Parkinsons disease.

The Prevalence and Characteristics of Charles Bonnet Syndrome in Turkish Patients with Retinal Disease

Purpose: The aim of this study is to investigate the prevalence and clinical characteristics of Charles Bonnet syndrome (CBS) in a group of Turkish patients with various retinal diseases. Methods: Two hundred and sixty-four patients with a best-corrected visual acuity of ≤20/40 in the better-seeing eye were asked with a standardized question whether they had symptoms of CBS. If they responded positively, a questionnaire was verbally administered to learn more about the details of the symptoms. Results: There were 125 (47.3%) females and 139 (52.7%) males with a mean age of 72.1 years (range 31-90). Seventeen (6.4%) patients were diagnosed with CBS. Three (17.7%) patients had noncomplex hallucinations and 14 (82.3%) had complex hallucinations. Conclusion: CBS is not uncommon in visually impaired patients with retinal disease. Clinicians who care for visually impaired patients should be aware of CBS.

Myoclonic status epilepticus in six patients without epilepsy

Myoclonic status epilepticus (MSE) is defined as prolonged period of myoclonic jerks that are correlated with epileptiform discharges on EEG. We here describe clinical features and video-EEG records of six adult patients with MSE who did not have a prior diagnosis of epilepsy. In four out of six patients, MSE was precipitated by drugs. Two out of four patients had chronic renal disease and received beta lactam group antibiotics. Two other patients, who described chronic pain, developed MSE while taking pregabalin. One patient who had dementia and family history of juvenile myoclonic epilepsy (JME) developed MSE one month after quetiapine was introduced. Another patient, who had a recent ischemic stroke, developed MSE due to an unknown reason. In these last two patients, an immediate triggering factor was not evident. Myoclonic status epilepticus ceased in five out of six patients after withdrawal of the drugs and/or intravenous antiepileptic treatment. Myoclonic status epilepticus is a rare event in patients without epilepsy. A correct diagnosis and prompt drug discontinuation may reverse this severe and life-threatening condition.

Turkish Standardization of Movement Disorders Society Unified Parkinson's Disease Rating Scale and Unified Dyskinesia Rating Scale

Movement Disorders Society Unified Parkinsons Disease Rating Scale (MDS‐UPDRS) and Unified Dyskinesia Rating Scale (UDysRS) were developed as standard tools to rate Parkinsons disease (PD) and drug‐induced dyskinesias of PD. As these scales have become widely used, there is a need for translation to non‐English languages. Here we present the standardization for the Turkish translations.

Assessment of voiding dysfunction in Parkinson's disease: Reliability and validity of the Turkish version of the Danish Prostate Symptom Score.

To investigate the reliability and validity of the Turkish version of the Danish Prostate Symptom Score (Dan‐PSS) questionnaire in patients with Parkinsons disease (PD) and to compare the burden of LUTS (Lower urinary tract symptoms) in men and women.

Swallow-induced syncope in 5 patients: Electrophysiologic evaluation during swallowing

Background: We sought to characterize a cohort of participants with swallow-induced syncope (SIS) with clinical and electrophysiologic evaluations. Methods: Using electrocardiographic monitoring and neurophysiologic methods of swallowing, we evaluated a cohort of 5 patients with SIS, 4 of whom had longitudinal follow-up. Results: We determined electrophysiologically that the duration between the onset of swallow and a bradyarrhythmia or asystole is extremely short (2–3 seconds) in SIS. Most participants with SIS do not have a neurologic or esophageal disorder. SIS can occur with different food types, in sitting or standing position, and has varying frequency in different participants. Permanent pacemaker placement is a curative measure in SIS. Conclusions: Our findings suggest that SIS is elicited by reflex afferent pathways originating in the oropharynx, rather than an esophageal origin, as previously proposed. Our longitudinally followed cohort with detailed clinical and electrophysiologic characterization should aid the clinician in the diagnosis and treatment of this potentially life-threatening condition.

P542: Electrophysiological evaluation of respiration-swallowing relationship in amyotrophic lateral sclerosis patients

Question: Amyotrophic lateral sclerosis (ALS) is a disease characterized by upper and lower motor neuron involvement. While lower motor neuron may be assessed by electrophysiological examination, upper motor neuron is required to be demonstrated clinically. Despite the recognized difficulty to evaluate upper motor neuron signs in patients with lower motor neuron involvement, transcranial magnetic stimulation (TMS) has not still been included in recent diagnostic criteria of ALS. We analyze the results of TMS in a group of ALS patients, and evaluate their sensitivity and predictive value. Methods: We retrospectively review patients with ALS studied in our laboratory during last 5 years. We selected those patients with transcranial magnetic stimulation performed during electrophysiological study. We evaluated amplitude of motor evoked potentials and central motor conduction time (CMCT) abnormalities. Results: Thirty patients with ALS were included in the study (range ages between 35 and 78 years), including sporadic and familial forms. Mild increase of CMCT was the most frequent abnormality in sporadic ALS patients, while severe increase was detected in familial ALS ones. Abnormalities of TMS preceded clinical pyramidal signs in some cases. Sensitivity and predictive diagnostic value were calculated. Conclusions: Transcranial magnetic stimulation is a useful method to assess upper motor neuron, and its implementation in the diagnosis of ALS can increase diagnostic sensitivity and proves to be a good predictor of corticospinal involvement.

Unilateral rhythmic testicular movements

A male patient came to our clinic because of a continuous up and down movement of his right testis, which was present even during sleep. The nature and localization of the unilateral rhythmic cremasteric activity is investigated by electrophysiological methods. Investigations of the cremasteric muscles and genitofemoral nerve determined that the movement was similar to myokymia. The localization of this peculiar spontaneous movement is probably in motor fibers somewhere between the L1-L2 motor neuron pool, motor roots and/or the genitofemoral nerve.