Bilateral papilledema caused by brucellosis mimicking pseudotumor cerebri.

In this article, we report a patient with migraine who was hospitalized with a prediagnosis of pseudotumor cerebri and diagnosed as neurobrucellosis with isolated intracranial hypertension presentation. A 22-year-old woman was admitted to emergency department with a complaint of headache. Her anamnesis indicated that she had migraine for 7 years. Neurological examination revealed bilateral papilledema. Cranial magnetic resonance imaging was normal. Cerebrospinal fluid (CSF) examination revealed 80 lymphocytes per mm3 with 178 mg/dL protein. Opening pressure was 260 mmH2O. Brucella tube agglutination and Rose Bengal tests were positive in blood and CSF. She was diagnosed as neurobrucellosis. If the systemic findings are insignificant and neurological findings are atypical such as isolated papillary edema, neurobrucellosis may not be considered and its diagnosis may be delayed. We believe that brucella serology should be included in the diagnostic protocols in endemic areas. Thus, early diagnosis and appropriate treatment can prevent complications of neurobrucellosis.

Ptosis and Elevation Paresis Due to Mesencephalic Infarction.

Lesions located in the anterior cavernous sinus or posterior orbit are known to cause divisional oculomotor palsy. We herein report a patient with fascicular infarction causing superior division paresis of the nerve.

Clinical and MRI findings of cerebellar agenesis in two living adult patients.

Cerebellar agenesis (CA) is an extremely rare entity. We present two adult patients with CA. The 61-year-old man had ataxia, dysarthria, abnormalities in cerebellar tests, severe cognitive impairment, and moderate mental retardation. The 26-year-old woman had dysmetria, dysdiadochokinesia, and dysarthria as well as mild cognitive impairment and mild mental retardation. Magnetic resonance imaging (MRI) showed complete absence of the cerebellum with small residual vermis. Brainstem was hypoplastic and structures above tentorium were normal. Supratentorial white matter bundles were unaffected in diffusion tensor tractography. Only few adult patients with CA have so far been published. These cases show that patients with CA present with a variety of developmental, clinical, and mental abnormalities; and emphasize the role of the cerebellum in normal motor, language, and mental development.

GNE Myopathy in Turkish Sisters with a Novel Homozygous Mutation.

Background. Hereditary inclusion body myopathy is caused by biallelic defects in the GNE gene located on chromosome 9p13. It generally affects adults older than 20 years of age. Methods and Results. In this study, we present two Turkish sisters with progressive myopathy and describe a novel mutation in the GNE gene. Both sisters had slightly higher levels of creatine kinase (CK) and muscle weakness. The older sister presented at 38 years of age with an inability to climb steps, weakness, and a steppage gait. Her younger sister was 36 years old and had similar symptoms. The first symptoms of the disorder were seen when the sisters were 30 and 34 years old, respectively. The muscle biopsy showed primary myopathic features and presence of rimmed vacuoles. DNA analysis demonstrated the presence of previously unknown homozygous mutations [c.2152 G>A (p.A718T)] in the GNE genes. Conclusion. Based on our literature survey, we believe that ours is the first confirmed case of primary GNE myopathy with a novel missense mutation in Turkey. These patients illustrate that the muscle biopsy is still an important method for the differential diagnosis of vacuolar myopathies in that the detection of inclusions is required for the definitive diagnosis.

Valproate-associated reproductive hormone abnormalities: do bipolar men have the same risk as epileptic men?

OBJECTIVE: The effects of valproate on male reproductive hormones have been studied in epileptic patients and animals, but the results are inconsistent because reproductive hormone abnormalities may be independent of the use of valproate and may be due to epilepsy itself. The aim of this study was to determine if there is an association between valproate and reproductive abnormalities in men with bipolar disorder or if the association is unique to men with epilepsy. MATERIALS AND METHOD: The study included 39 male patients aged 18-50 years with a DSM-IV diagnosis of bipolar disorder (21 on lithium monotherapy and 18 on valproate monotherapy or valproate in combination with lithium therapy) and 15 male epilepsy patients on valproate monotherapy that were evaluated in terms of reproductive hormones. RESULTS: Duration of illness, duration of lithium and valproate therapy, daily dose and serum concentrations of lithium and valproate, duration of marriage, spouse's gravidity, the serum estradiol, luteinizing hormone, sex hormone-binding globulin, and free testosterone levels, and the free testosterone:luteinizing hormone ratio were not significantly different between the groups. Serum prolactin and follicle-stimulating hormone levels were significantly higher in the epilepsy patients than in the bipolar disorder patients on lithium monotherapy. CONCLUSION: The findings show that valproate did not have a negative effect on male reproductive hormones in the bipolar patients. The elevated prolactin and follicle-stimulating hormone levels observed in the epilepsy group should be attributed to epilepsy. To the best of our knowledge this is the first study to compare reproductive hormones in bipolar disorder and epilepsy patients on valproate therapy.

Psychiatric comorbidity, quality of life and social support in epileptic patients.

BACKGROUND: Epilepsy is accompanied by many psychiatric disorders and continues to be a disease that disturbs the functioning and quality of life (QOL) of patients. AIMS: To assess the frequency of psychiatric disorders occurring in epileptic patients, determine depression and anxiety levels, and compare the results with healthy controls. The effect of epilepsy on QOL and the effects of social support on epilepsy were also assessed. METHODS: 50 epileptic patients and 50 controls were compared using the Structured Clinical Interview for DSM-IV (SCID-I), Beck Depression Inventory, Beck Anxiety Scale, Multi-dimensional Scale of Perceived Social Support (MSPSS) and the World Health Organization Quality of Life Assessment-Brief (WHOQOL-Brief). RESULTS: 44% of epileptic patients had psychiatric disorders, with depression being the most common diagnoses (28%). In terms of psychiatric comorbidity, there was a significant difference between the groups. The depression and anxiety scores of epileptic patients were significantly higher and there was no difference between the groups in terms of MSPSS scores. Psychological and physical subscale scores of WHOQOL-Brief were significantly lower in epileptic patients. In the epileptic group, a multi-variate analysis of covariance demonstrated that depression was a significant covariate for physical and psychological domains, anxiety was a significant covariate for psychological domain, social support was a significant covariate for social and environmental domains, and age of epilepsy onset was a significant covariate for physical domain of QOL. CONCLUSION: Epilepsy needs multi-dimensional analysis because of its chronic nature, frequently accompanying psychiatric disorders, its negative effects on QOL and the important role social support plays. The clinicians' handling of patients at an early stage can play an important role in preventing the negative results of epilepsy.

Recovery of visual-field defects after occipital lobe infarction: a perimetric study.

OBJECTIVE: To assess the temporal course of homonymous visual-field defects due to occipital lobe infarction, by using automated perimetry. METHODS: 32 patients with ischaemic infarction of the occipital lobe were studied prospectively, using a Humphrey Visual Field Analyser II. The visual field of each eye was divided into central, paracentral and peripheral zones. The mean visual sensitivity of each zone was calculated and used for the statistical analysis. The results of the initial examination, performed within 2 weeks of stroke, were compared with the results of the sixth-month control. The lesions were assigned to the localisations, optic radiation, striate cortex, occipital pole and occipital convexity, by MRI. RESULTS: A statistically significant improvement was noted, especially for the lower quadrants. Lesions of the occipital pole and convexity were not significantly associated with visual-field recovery. However, involvement of the striate cortex and extensive lesions involving all the areas studied was significantly associated with poor prognosis. CONCLUSIONS: Homonymous visual-field defects in our patients improved within 6 months. Restoration of the lower quadrants and especially the peripheral zones was noted. Incomplete damage to the striate cortex, which has a varying pattern of vascular supply, could explain this finding. Magnification factor theory, which is the increment of the receptive-field size of striate cortex cells with visual-field eccentricity, may explain the more significant improvement in the peripheral zones.

Clinical and radiologic correlations of small and large centrum ovale infarcts.

BACKGROUND: Acute centrum ovale (CO) infarction confined to the territory of perforating long medullary arteries is rarely reported. This study was designed to investigate the full spectrum of clinical findings, topography, and pathogenesis of patients with acute CO infarct. METHODS: A total of 64 patients with CO infarct were recorded. Risk factors such as age, hypertension, diabetes mellitus, hypercholesterolemia, smoking, and cardiopathy were recorded. RESULTS: The average age of the patients was 64.2 years. There were 46 patients (71.8%) in large-infarct group and 18 patients (28.1%) in small-infarct group. The most frequent risk factor was hypertension (89.1%). Other risk factors included smoking (39%), diabetes mellitus (29.7%), hyperlipidemia (28.1%), transient ischemic attack (15%), carotid stenosis (9%), atrial fibrillation (21%), other potential cardiac source of embolism (39%), and myocardial infarct (4%). The main clinical feature was faciobrachiocrural paralysis associated dysarthria (51.6%). Other clinical characteristics were facial, upper and lower extremity paralysis with sensorial deficits and/or dysarthria (26.6%), hemiparesis including face (12.5%), and either facial and upper or upper and lower extremity paralysis with hemisensorial deficit (9.4%). The baseline Barthel index was found to be less than 50 in 22 patients (34.4%) and greater than 50 in 42 patients (65.6%) for the entire group whereas 72% of patients were independent at discharge. CONCLUSION: In this article, risk factors, clinical spectrum, and features of CO infarcts were correlated in a large patient group. The majority of CO infarcts were large and had a poorer outcome compared to previous reports.

Hemifacial spasm in correlation with electrophysiological and radiological findings.

OBJECTIVE: To investigate the correlation between the electrophysiological and radiological findings of primary hemifacial spasm patients. SUBJECTS: Patients with primary hemifacial spasm who had had no botulinum toxin treatment previously were included in the study. DESIGN: In this prospective study patients underwent cerebral magnetic resonance imaging,a magnetic resonance angiography investigation and an electrophysiological examination after informed consent had be given by the patients. The facial nerve distal latency, amplitude and blink reflex responses were recorded as well as clinical and demographic data. RESULTS: Twenty five patients completed radiological and electrophysiological investigations. The radiological investigations disclosed neurovascular compromise which can cause hemifacial spasm in twelve patients (48%) while the findings of two patients were not considered as a certain cause of hemifacial spasm. All patients except two had at least one electrophysiological abnormality (92%). The most frequent finding was an increased R1/D ratio which suggested an increased central conduction time. CONCLUSION: In this study, an increased R1/D ratio suggests that there is a functional impairment in the brain stem even if it is not possible to disclose structural abnormalities in some hemifacial spasm patients. Combining magnetic stimulation may be a useful tool for further investigations. An inadequate radiological investigation might be the cause of relatively low radiological abnormalities in comparison with the electrophysiological ones.

Isolated hand palsy due to cortical infarction: localization of the motor hand area.

OBJECTIVE: To evaluate the cortical presentation of the hand area according to MRI images of the infarcted area in patients who have predominantly hand weakness. BACKGROUND: "Pseudoperipheral palsy" is an old term employed to describe a rare clinical picture consisting of predominant weakness of the hand in association with cerebral infarction. Because the organization of the cortical efferent area of the hand has not been fully defined, this phenomenon merits attention. Recent advances in functional imaging have stimulated renewed interest in the cortical mapping of various body parts, particularly that of the hand. METHODS: The patients who had isolated hand palsy due to cerebral infarction underwent clinical examination and cerebral MRI investigation to locate the exact lesion site. RESULTS: Eight patients (6 men, 2 women) aged 55 to 80 years (mean 69 years) were included in this study. All had at least one risk factor for cerebrovascular disease. All our 8 patients presented with hand palsy and lesions were detected in the contralateral precentral gyrus in all cases. The lesions were located in the middle to lower portion of the anterior wall of the central sulcus just posterior to the intersection of the superior frontal and precentral sulci. CONCLUSION: Our findings indicate that the hand area in the cerebral motor cortex is located in the middle to lower portion of the anterior wall of the central sulcus, that is, in Brodmann area 4. We present our 8 patients showing isolated hand palsy due to a discrete cortical infarction on MRI to expand the knowledge about cortical localization of the hand area.

Dysarthria due to supratentorial and infratentorial ischemic stroke: a diffusion-weighted imaging study.

BACKGROUND AND PURPOSE: Dysarthria characterized by slurring with imprecise articulation without evidence of aphasia is a frequent symptom in the acute phase of cerebral ischemia, although there is little knowledge on its anatomic specificity and spectrum of associated clinical characteristics regarding diffusion-weighted imaging (DWI). METHODS: An investigation of 101 consecutive patients with sudden-onset dysarthria due to a single or multiple lesions on DWI, corresponding to 8.7% of 1,160 patients with ischemic stroke, was made. The presence of lesions of the cranial arteries was sought by magnetic resonance angiography and reviewed with a three-dimensional rotating cineangiographic method. RESULTS: Dysarthria was mostly associated with a supratentorial lesion (63%) and with a classic lacunar stroke syndrome in 45% of patients. Lacunar lesions on DWI were found in 69 (68%) patients, while only 45 of the patients (65%) with a lacunar infarct presented a classic lacunar syndrome with dysarthria. Pure dysarthria occurred in 15% of patients, dysarthria + pure motor hemiparesis in 14%, dysarthria + ataxic hemiparesis in 11%, dysarthria + clumsy hand syndrome in 7%, dysarthria + pure sensory stroke in 3%, dysarthria + central facial paresis in 8% and lingual paresis occurred in 2%. The lesions were due to small-artery disease in 41%, large-artery disease in 15%, cardioembolism in 10% and a mixed etiology in 3%. The cause of stroke was not identified in 17 patients. Lesions on DWI were found mainly in the corona radiata (n = 18), middle cerebral artery territory, including the motor cortex and/or insular cortex (n = 13), striatocaudate nuclei (n = 11), primary motor cortex (n = 10), internal capsule (n = 7), pons (n = 25), pontobulbar junction (n = 5) and the thalamomesencephalic junction (n = 4). Isolated cerebellar infarctions (n = 6) or associated brainstem lesions (n = 6) affected mostly the superior cerebellar artery or the posterior inferior cerebellar artery territory. CONCLUSION: Cortical involvement was more frequent in patients with pure dysarthria than those with dysarthria and additional neurological signs, while the frequency of pontine involvement was higher in patients with additional neurological signs than those with pure dysarthria. One third of the patients with dysarthria had multiple lesions on DWI, and the most common cause of stroke was small-artery disease. Pure dysarthria, dysarthria with lingual paresis, dysarthria with clumsy hand and dysarthria with facial paresis had predictive value for lacunar lesions.

Interaction of botulinum toxin type A with local anesthetic agents: an experimental study with rabbits.

Injection of botulinum toxin is a painful procedure, limiting the number of patients treated. This study was designed to establish whether infiltration of local anesthetics plays a role in the effectiveness of botulinum toxin. For the study, 24 New Zealand white rabbits were divided into three groups. In all three groups, local anesthetics (prilocaine, lidocaine, lidocaine, and epinephrine) were injected into the right anterior auricular muscle before a 2.5-U injection of botox A. The contralateral anterior auricular muscle of all the rabbits was injected with the same dose of the toxin to serve as a control. Photographic documentation was recorded; electromyographic study was performed; and results were statistically analyzed. The results show that local anesthetics do not play a role in the efficacy of botulinum toxin. Thus, the use of infiltrative local anesthetics before botulinum toxin injection is a safe method for achieving a less painful procedure for the patient.

Isolated dysarthria-facial paresis syndrome: a rare clinical entity which is usually overlooked.

BACKGROUND AND AIMS: The aim of the study is to search the lesion localization of the pure isolated facial paresis-dysarthria syndrome in patients who were admitted to our neurology clinic in a prospective study. METHODS: Over a period of six years, the patients who had no prominent sensorimotor dysfunction were examined by neurologists and underwent computerized tomography (CT) and/or magnetic resonance imaging (MRI). RESULTS: Eleven patients out of more than 2000 had the aforementioned clinical picture. Lacunar infarctions were identified at the corona radiata in nine patients, and at the internal capsule in two patients. As reported previously, facial paresis was usually mild and temporary. Six of our eleven patients were seen at the outpatient clinic one month later. Four of them had completely recovered and the other two had mild dsyarthria without any facial paresis. The other five could not be reached after leaving the hospital. CONCLUSIONS: Dysarthria-facial paresis is a rare clinical entity and possibly a variation of dysarthria-clumsy hand syndrome, and we suggest that pure facial paresis (FP) and pure dysarthria should be considered as very extreme examples of this syndrome.