Right cerebral hemiatrophy: neurocognitive and electroclinical features.

The purpose of this study was to retrospectively evaluate the cognitive and electroclinical characteristics of right cerebral hemiatrophy (Dyke-Davidoff-Masson syndrome [DDMS]). Cognitive assessments with a particular emphasis on visuospatial functions, electroclinical features, and neuroimaging characteristics were analyzed for five patients with a clinically and neuroradiologically confirmed diagnosis of right-sided DDMS. Intelligence tests revealed mental retardation in all but one. Neuropsychological assessments demonstrated consistent impairments in tasks that have a spatial component (spatial processing and orientation discrimination), whereas attention, executive functions and verbal memory domains were variably impaired. Electroclinically, the main seizure types were simple partial motor, complex partial, and secondarily generalized seizures. Interictal EEG delineated lower amplitudes and slow background activity in the affected hemisphere. Overall, the cognitive performance of patients with DDMS encompasses a broad spectrum of impairments affecting multiple domains. Our findings support the concept that dorsal visual pathways responsible for spatial processing may be lateralized to the right hemisphere.

Frontal assessment battery in patients with Parkinson disease in a Turkish population.

OBJECTIVE: The aims of this study were to assess frontal dysfunction in Turkish patients with idiopathic Parkinson disease (PD) by using frontal assessment battery (FAB), and to determine the relationship among FAB scores and education, Mini-Mental State Examination (MMSE), and severity of the disease. BACKGROUND: Cognitive impairment in patients with PD mainly involves executive dysfunction. Executive dysfunction is mainly related to the frontal lobes and their connections with the thalamus and basal ganglia. FAB is an easily applicable and valid test to examine frontal functions. METHOD: Forty patients diagnosed with PD and 52 healthy controls were included in the study. Age, sex, disease duration, and Unified Parkinson's Disease Rating Scale scores were noted. FAB and MMSE were administered to all participants. Both groups were compared according to FAB scores, MMSE, age, and education. RESULTS: FAB scores were significantly lower in patients with PD (P=0.00) than in healthy controls. In patients with PD, FAB scores were solely correlated with MMSE. In the subgroup analysis, the performance of patients with PD was worse than the healthy participants on similarities and fluency tasks. CONCLUSIONS: FAB scores are lower in Turkish patients with PD than in the healthy participants, and could be used for the evaluation of subtle cognitive deficits in these patients.

Cerebral microbleeds in ischemic stroke patients on warfarin treatment.

BACKGROUND AND PURPOSE: Cerebral microbleeds (CMBs) are known to be indicative of bleeding prone microangiopathy. Little is known about its significance in anticoagulated patients. We aimed to determine the frequency of CMBs in ischemic stroke patients on warfarin treatment. METHODS: A total of 141 ischemic stroke patients on warfarin therapy were enrolled in this study. One hundred five patients with similar demographic features who do not use warfarin were chosen as controls. We compared vascular risk factors and radiological findings including CMBs and leukoaraiosis between the groups. RESULTS: CMBs on gradient-echo MRI (GE-MRI) were found in 31 patients (22%) and 17 controls (16%) and there was not a significant difference between 2 groups (P=0.25). Study patients with CMBs were older than patients without CMBs (P=0.04) and frequency of leukoaraiosis was significantly higher (P=0.008). Mean duration of warfarin treatment was not different between the patients with and without CMBs (P=0.83). CONCLUSIONS: Although patients with CMBs were older and had more leukoaraiosis the impact of warfarin treatment on CMBs is still controversial.

Intrathecal methotrexate-induced acute chorea.

Intrathecal methotrexate is the mainstay of central nervous system prophylaxis in acute lymphoblastic leukemia. We describe a patient who developed acute chorea after last dose of intrathecal methotrexate therapy and recovered completely.

Panayiotopoulos syndrome with coincidental brain lesions.

PURPOSE: To describe the clinical and electroencephalographic features of three patients diagnosed with Panayiotopoulos syndrome with different lesions identified by cranial MRI investigation. METHODS: Our study was based on patients from the Epilepsy Outpatient Clinic of Sisli Etfal Education Hospital in Istanbul, where a prospective study of Panayiotopoulos syndrome was initiated in 1995. Records of our Epilepsy Outpatient Clinic revealed 53 patients with Panayiotopoulos syndrome. Among them were three with cranial lesions identified by MRI. RESULTS: The onset of the seizures in our patients ranged between five and eight years. The seizures included mainly autonomic symptoms such as nausea, vomiting, pallor, mydriasis, urinary and fecal incontinence, and rarely hypersalivation. Autonomic partial status was detected in one patient. The personal history of our patients revealed head trauma in two and difficult birth history in one patient. Two patients described simple febrile seizures. All patients had occipital spike or spike-wave complexes in their EEGs. The background activity was normal. From the cranial MRI, one patient had a neuroepithelial cyst, the second patient had a right occipital encephalomalasic lesion and the third patient had an arachnoid cyst located in the cisterna magna associated with colpocephaly. Seizure frequency was low in general. All patients except for one received carbamazepine treatment 450-600 mg/day. None of our patients had seizures under antiepileptic treatment. CONCLUSIONS: Children with Panayiotopoulos syndrome may have static MRI brain findings which are likely to be coincidental and do not affect prognosis.

Plasma cortisol levels in migraineurs between attacks.

We have studied the plasma cortisol levels between attacks in order to investigate the role of hypothalamic-pituitary-adrenal axis in migraine patients. We have not found any statistically significance between plasma cortisol levels of patients and control group, after taking the blood of 25 patients and 24 healthy controls between 8.30-9.30 am.

Prevalence of patent foramen ovale in patients with migraine.

Recent evidence supports that the prevalence of patent foramen ovale is higher in patients with migraine with aura. We conducted a case-control study and searched for intra-atrial right to left shunt in 53 patients with migraine. PFO was detected by means of transthoracic echocardiography with administration of contrast medium during valsalva maneuver and the results were compared with age and sex matched 27 healthy controls. Patent foramen ovale was more frequent in the migraine group (p<.01). The percentages of PFO in migraine patients with aura, without aura and the control group were 66.7%, 47.4% and 22.2%, respectively. Our results are supportive of an association between PFO and migraine, especially with aura.

Overlap cases of eyelid myoclonia with absences and juvenile myoclonic epilepsy.

Eyelid myoclonia with absences (EMA) and juvenile myoclonic epilepsy (JME) are two separate epileptic syndromes included in the new classification of epilepsies and epileptic syndromes by ILAE in 2001. Both are idiopathic generalized epilepsies with their clinical onset in the first two decades. EMA is characterized by eyelid myoclonia associated with absences and photosensitivity. Self-induced seizures are frequently seen in EMA. It can be associated with mildly mental retardation and resistance to treatment. JME includes three types of generalized seizures: typical absences, myoclonic jerks and generalized tonic-clonic seizures. The myoclonic jerks occur almost exclusively on awakening, involve preferently the upper extremities, may rarely affect the lower extremities or the entire body. More severe attacks may be accompanied by a fall. The myoclonic jerks occur rarely in EMA. They are usually mild and are freqently restricted to the upper extremities. Generalized tonic-clonic seizures, photosensitivity and generalized polyspike-wave discharges provoked by eye closure are features of both epileptic syndromes. In this study, we describe four female patients with eyelid myoclonia associated with absences, myoclonic jerks causing falling down and rare generalized tonic-clonic seizures. All patients had good school performance and total seizure control under sodium valproate treatment. Their EEGs show generalized polyspike-wave discharges with a frequency of 3.5-6Hz always appearing a few seconds after eye closure and photoparoxysmal response. These patients show the characterictics of both epileptic syndromes. It is clinically important to make a syndromic diagnosis for an optimum advise on treatment, lifestyle restrictions and prognosis. In this study, we have gathered evidence that EMA and JME are dynamic syndromes that tend to evolve into one another.

A comparison between rate of nonmotor symptom development in essential tremor and Parkinson's disease.

In the last decade our perspective on essential tremor (ET) as a pure motor system disorder has begun to change. By virtue of recent studies of nonmotor symptoms (NMSs) that are used to characterize Parkinson's disease (PD), these symptoms have also been added to the definition of ET. There is increasing evidence to suggest that ET might not be as benign and monosymptomatic as we previously thought. The aim of this study was to evaluate nonmotor symptoms in ET, and to compare them with PD. We studied 37 ET and 23 PD patients. Tremor rate was evaluated using the Fahn-Tolosa-Marin tremor rating scale (FTM-TRS) in ET patients. The patients with PD were scored for motor symptoms using the unified Parkinson's disease rating scale (UPDRS)-III and the Hoehn-Yahr scale. Cognitive functions were assessed with the Montreal Cognitive Assessment (MoCA) test. NMSs were evaluated with the nonmotor symptoms questionnaire (NMSQuest). In the ET group, the most common NMSs were forgetting things, feeling sad, nocturia, urgency, and difficulty concentrating. The mean NMSQuest score was 8.43 ± 4.14 in the ET group and 14.06 ± 5.44 in the PD group (p value <0.001). However, except for 12 items in NMSQuest, in comparing items one by one there was no statistical difference between them. The mean MoCA total score was 17.81 ± 4.56 in the ET group and 17.08 ± 4.08 in the PD group (p value 0.675). There were no significant differences in MoCA subgroup scores. Evaluation of nonmotor symptoms in ET may help us to understand this emerging definition of ET. This study contributes evidence toward this new concept.

Evidence against association between arachnoid cysts and epilepsy.

Arachnoid cysts are often discovered incidentally in MRs performed for a variety of reasons. In this study, we have attempted to determine a possible relationship between the arachnoid cyst, seizure type and EEG abnormalities. Eight patients were classified according to the Classification of Epilepsies and Epileptic Syndromes of ILAE as idiopathic generalized or localized epilepsy: three as idiopathic generalized epilepsy, three as Rolandic epilepsy and two as juvenile myoclonic epilepsy. Two patients with rare nocturnal seizures had normal EEGs in the awaking and sleep stage. The EEGs of five patients with simple or complex partial seizures revealed focal epileptiform abnormalities, but only one patient had the same location with the arachnoid cyst. Five patients with symptomatic epilepsy had diffuse slowing in their EEGs and only one patient had seizure focus in the EEG that was contralateral to the arachnoid cyst. According to our study, seizure type and EEG abnormality corresponds to arachnoid cyst location in only one patient. Therefore, we suggest that arachnoid cysts may not be related to a specific seizure type and EEG focus.

Involuntary movements associated with vitamin B12 deficiency.

Involuntary movements are not a known feature of vitamin B12 (vB12) deficiency in adults, though they are a characteristic feature of vB12 deficiency in infants. This case report presents an adult patient with vB12 deficiency in whom, myoclonus-like muscular contractions appeared soon after the initiation of vB12 and disappeared after the first week of this therapy. To our knowledge, this is the first report of involuntary movements in an adult patient with vB12 deficiency. Although the mechanism remains unknown, involuntary movements similar to myoclonus should be considered as one of the extraordinary neurological manifestations of vB12 deficiency in adults.

Lack of association between cerebral microbleeds and low serum cholesterol in patients with acute intracerebral hemorrhage.

BACKGROUND: The association of cerebral microbleeds (CMBs) with intracerebral hemorrhage (ICH) is well known and its relationship with low serum cholesterol in ICH patients might be of interest. METHODS: A total of 105 patients with ICH were evaluated. In all subjects cholesterol levels were measured after 12h of fasting and gradient-echo magnetic resonance imaging (GE-MRI) was performed for detecting CMBs. RESULTS: CMBs were more common among patients with hypertension and leukoaraiosis (p=0.008 and p=0.001). Patients with and without CMBs did not differ according to total cholesterol, LDL cholesterol, triglycerides and HDL cholesterol levels. CONCLUSION: In this study, 61% of Turkish ICH patients had CMBs, which was not associated with lipid profiles. Leukoaraiosis was independently associated with CMBs.

The relation of testosterone levels with fatigue and apathy in Parkinson's disease.

OBJECTIVE: Fatigue and apathy are frequent in patients with Parkinson's disease (PD). Testosterone deficiency in male patients may contribute in development of fatigue and apathy as well. We investigated whether a possible relation exists between serum testosterone levels, fatigue and apathy in male PD patients. MATERIALS AND METHODS: We included 29 non-demented and non-depressed PD patients and 30 age- and sex-matched healthy subjects. Fatigue Severity Scale (FSS) and Apathy Evaluation Scale (AES-C) were used for the evaluations. In PD patients and healthy subjects, a relationship between FSS, AES-C scores and plasma testosterone levels were assessed. In addition, a correlation between FSS, AES-C and Unified Parkinson's Disease Rating Scale was investigated in PD group. RESULTS: The mean scores of FSS and AES-C were significantly higher in PD patients than those of the control group. The Unified Parkinson's Disease Rating Scale (UPDRS) scores were significantly correlated with FSS and AES-C scores. Mean free testosterone level was significantly lower in PD patients than controls (p=0.008). f-Testosterone levels of PD patients were not correlated with FSS or AES-C scores. CONCLUSION: Apathy and fatigue are frequent in PD and show significant correlation with the severity of the disease. f-Testosterone levels are not related with apathy or fatigue in male PD patients and the role of testosterone in the pathophysiology of these non-motor symptoms is still controversial.

Prevention of deep venous thrombosis and pulmonary embolism in patients with acute intracerebral hemorrhage.

OBJECTIVES: Although patients with intracerebral hemorrhage (ICH) are at risk for deep venous thrombosis (DVT), the data about preventive options for DVT prophylaxis in this population is insufficient. We investigated the safety of low dose low molecular weight heparin (LMWH) for DVT prophylaxis in patients with ICH and the effect of heparin on the enlargement of hemorrhage. METHODS: We prospectively randomized 75 primary ICH patients to subcutaneous LMWH (Enoxaparin sodium 40mg/d) or long compression stockings (CS) after the first 48 hours. All patients had cranial computed tomography (CT) scan at admittance, 24th and 72nd hours, seventh and 21st days, CT pulmonary angiography and bilateral lower extremity venous Doppler at 7th day. Hematoma volumes were calculated on the initial and follow-up CTs with ABC/2 method. RESULTS: Mean +/- SD age of the patients was 68.1 +/- 11.98 and 66.08 +/- 9.55 in LMWH and CS groups, respectively. Twenty-two of LMVH group and 8 of CS group were female. After randomization to LMWH or CS, we did not observe any hematoma enlargement at 72nd hours, 7 and 21st days in both groups. In addition, there was not any other systemic bleeding complication in LMWH group. We detected 4 asymptomatic DVT in our patients (3 in LMWH and 1 in CS group). Although asymptomatic DVT was more common in LMWH group, it was not statistically significant (P = 1). CONCLUSIONS: Low dose heparin treatment after 48 hours of stroke in ICH patients is not associated with an increased hematoma growth and should be used for DVT and PE prophylaxis.

Triphasic EEG pattern in bilateral paramedian thalamic infarction.

Two cases of bilateral paramedian thalamic infarction (BPTI) showing triphasic waves (TWs) on the electroencephalogram (EEG) at acute stage are presented in this study. BPTI is a rare syndrome with decreased level of consciousness, gaze abnormalities and cognitive deterioration. TWs are nonspecific EEG findings occurring in both metabolic and nonmetabolic conditions. The TWs in BPTI might be related to level of consciousness and does not always predict a poor prognosis in BPTI.

Hot water epilepsy: clinical and electroencephalographic features of 25 cases.

The aim of this study was to analyze the clinical and electroencephalographic findings for 25 patients with hot water epilepsy. Personal and family history, neurological state, age at onset and types of seizures, provoking factors, bathing habits, EEGs, and neuroimages were all reviewed. Age at onset of seizures ranged from 6 months to 37 years. Twenty patients had complex partial seizures. Eight patients had spontaneous seizures as well; one was a typical case of idiopathic photosensitive occipital lobe epilepsy. One patient described an episode interpreted as nonconvulsive status. In our study group, the pouring of the water over the head and the temperature of the water were the most common triggering factors. A special kind of soap and entry of water into the mouth were determined to be unusual triggering factors. Interictal EEGs revealed epileptogenic abnormalities located over the temporal regions in nine patients. Seventeen patients underwent neuroimaging, mostly cranial magnetic resonance imaging. One had right mesial temporal sclerosis and one cortical atrophy; the others had normal findings. Sixteen patients received antiepileptic drugs, mainly carbamazepine, and remained seizure-free. The high rate of epileptogenic abnormalities localized in the temporal region and the complex partial seizures observed in most of our patients indicate the considerable role of the temporal lobe in hot water epilepsy.