Neurologische Störungen

Background: Multiple sclerosis (MS) is a chronic, degenerative autoimmune inflammatory disease leading to demyelination and axonal damage to the brain and spinal cord. This entity is low prevalence in Ecuador, and so far there are few reports regarding this disease. Objective: To establish clinical and epidemiological characteristics of MS patients at Hospital Carlos Andrade Marín (HCAM) in the city of Quito, and to compare these findings with the reports published worldwide. Methods: Using the McDonald criteria for diagnosis of MS 2010, we retrospectively reviewed the medical records of patients with MS who came to HCAM in Quito from January 2005 to December 2014. Degree of disability information, as well as clinical and demographic data was recorded. Then, a descriptive analysis of the data was performed. Outcomes: 102 cases of multiple sclerosis were identified, the majority of which were female (female to male ratio of 2.2:1). The average age of this population was 39.6 years (SD +/-11.6), and the average age of symptom onset was 32 years (SD +/-11.1). The majority of cases corresponded to the recurrent remittent multiple sclerosis (RRMS) type at 93%. Only 3 were progressive MS cases and 8 cases represented clinically isolated syndromes (CIS). Among the initial clinical manifestations, optical neuritis (ON) was present in 33% of cases, acute myelitis (AM) in 60% and 12.7% of patients debuted with brainstem manifestations. Motor, visual and sensory symptoms were the most common clinical manifestations established (79.67% and 63.7%, respectively). Cognitive and neuropsychiatric disorders were also common (56% and 37%, respectively). The average disability measured by The Expanded Disability Status Scale (EDSS) was 3.7. Conclusion: This is the first descriptive study of a cohort of patients with MS from the City of Quito, Ecuador. The clinical and epidemiological characteristics of this cohort did not differ from those described worldwide.

Introduction: According to the ILAE, the definition of epilepsy requires the occurrence at least one epileptic seizure. Up to 20% of patients diagnosed of epilepsy are not actually epileptic. An accurate diagnosis is necessary for a satisfactory management of patients with Epilepsy. New broad access image technologies allow patients and families to record homemade videos helping neurologists in diagnosis.

Method: During a two-year period in an epilepsy clinic, consecutive patients were encouraged to record their events with any available device. Instructions for good quality videos were given. Three neurologists/epileptologists watched the videos in clinical session, rated the quality of the recordings following some parameters and made a clinical diagnosis. In a second phase, previous diagnosis was revised.

Results: 314 consecutive patients (relatives) were encouraged to record events. 52% male. Average age: 46 years. 267 patients had video recording devices available (87%) (Photo camera: 100%, cell-phone: 100%, webcam: 10%, video camera: 30%). From this group, 135 (50%) felt unable to record events. Reasons given: Low seizure frequency: 60%, seizures short duration: 80%. 50 events from 22 patients recorded. Mean age: 35 years. Seizure frequency three months prior to video deposit was 3, 5 seizures/patient/month. Previous epileptic syndrome diagnosed (based on description/neuroimaging/EEG): 15 focal temporal lobe epilepsy, 4 focal frontal probably symptomatic epilepsy, 3 epileptic encephalopathy.

Type of seizures recorded: Focal motor with typical automatisms: 14 seizures/11 patients. Focal motor with hyperkinetic automatisms: 9 seizures/2 patients. Asymmetrical tonic motor seizures: 4 seizures/1 patient. Focal clonic seizure: 5 seizures/1 patients. Atypical absence seizure: 6 seizures/3 patients. Non-epileptic Seizure (NES): 13 seizures/3 patients. Postural tremor: 1 patient. There was agreement in diagnosis but in one. 18 patients were confirmed in their Diagnosis: Epilepsy misdiagnosis: 4. Three NES and one undetermined tremor.

Conclusion: Homemade videos may be of diagnostic value in epilepsy management. Training in performing good-quality videos is necessary. Webcam long term recordings should be recommended as the best recording option.

Herpes Simplex encephalitis is eminently treatable viral encephalitis which can rarely be complicated by a tumefactive inflammatory focus. The treatment strategy in this event should be aggressive to prevent immediate fatality as well as long term sequele. A 43-year-old woman with HSE developed new onset focal deficit with altered sensorium on the fourth day of illness despite early initiation of antiviral treatment. Imaging revealed right temporal and insular involvement with mass effect and midline shift. Decompressive craniectomy and temporal lobe biopsy was performed on emergent basis. The patient made remarkable clinical recovery subsequently. The report is presented for highlighting this rare complication as well as to reinstate the belief in surgical decompression as the preferred choice of treatment.

Gerstmann's syndrome is a neuropsychological disorder characterized by four symptoms, namely, acalculia, finger agnosia, left-right disorientation, and agraphia suggesting the presence of a lesion in the inferior parietal lobule of the dominant hemisphere, especially at the angular gyrus. Several descriptions of Gerstmann's syndrome have been reported in associated with a lesion to the left frontal lobe, but none of these reports fulfilled the full tetrad of diagnostic criteria. Herein, we report two cases with all four symptoms of Gerstmann's syndrome associated with an uncommon area in the left middle frontal lobe without an angular gyrus lesion. This apparent discrepancy could be explained by disconnection of functional fiber between the frontal and parietal cortex. Moreover, left frontal cortex or the subcortical area may be an important functional area which is closely connected with the parietal lobe in Gerstmann's syndrome.

Background and objective: To analyze the temporal evolution of imaging findings and clinical follow up in craniocervical arterial dissection (CAD) patients. Methods: 32 patients (M: F=23: 9, mean age=49) with radiographic evidence of at least one of the suggestive imaging findings of CAD (arterial stenosis, arterial occlusion, luminal ectasia, pseudoaneurysm, and dissection flap) were performed. Median imaging follow up period was 89 days (SD=36.69). Clinical symptoms on presentation and follow up were also analyzed. Results: 21 patients had spontaneous dissection (65.6%). 11 had a history of trauma (34.4%). 14 (43.8%) patients presented with a stroke or TIA. Other presenting symptoms include headache (n=4, 12.5%), neck pain (n=9, 28.1%) and Horner’s syndrome (n=5, 15.6%). Involved arteries included Internal carotid artery (n=21, 65.6%), vertebral artery (n=10, 31.3%), and common carotid artery (n=3, 9.4%). Initial imaging findings included, arterial stenosis (n=23, 72%), arterial occlusion (n=6, 19%), ectasis of the lumen (n=7, 22%), pseudoaneurysm (n=12, 38%), and dissection flap (n=12, 38%). 21 patients were managed with anti-coagulation and 3 with an antiplatelet agent. On follow up imaging, arterial stenosis was improved in 73.6% (14/19), worse in 5.3% and no interval change in 21.1%. Regarding the pseudoaneurysm on presentation, no change in shape and size in 64% (7/11) and 2 patients developed new pseudoaneurysm. One patient developed a TIA (3.7%, n=1/27) during follow up. Conclusion: Post-dissection vascular imaging findings are dynamic. With medical management, more than 60% of the arterial stenosis lesions have improved within 3 months, and the risk of repeated neurological events were very low.

Because of their anatomic proximity, pupillary and inferior rectus functions are linked in most cases of fascicular oculomotor palsy, with either sparing or involvement of both. A 40-year-old woman presented with painless binocular diplopia and left ptosis. Examination additionally showed limitations of the adduction and depression in the presence of normal supraduction and intact pupillary function in the left eye.

Findings of other neurological neuro-ophthalmologic examination were normal. A magnetic resonance imaging revealed a diffusion restriction in the left dorsomedial midbrain. An ischemic lesion restricted to the oculomotor fascicles can cause inferior rectus palsy in the presence of pupillary sparing, which should be differentiated from a microvasculopathic lesion involving the subarachnoid portion of the oculomotor nerve.

The Authors report a new technique with trans sacral epiduroscopy assisted Percutaneous Endoscopic Lumbar Discectomy (PELD) for high grade centrally down migrated lumbar disc herniation. When the chances of missing the disc fragments are high, as in the cases of down migrated disc herniations by conventional transforaminal PELD, it can be assisted by sacral epiduroscopy for the complete removal and confirmation of any remnant disc fragments.

Introduction: The cause for fibromyalgia (FM) is unknown and diagnostic criteria can be nonspecific. Many patients with FM have nonspecific sensory symptoms consistent with a neuropathic process. Previous studies have shown that a significant percentage of patients diagnosed with FM have small fiber neuropathy (SFN) based on decreased intraepidermal nerve fiber density (IENFD) on according to punch skin biopsy testing. The purpose of this study was to demonstrate that punch skin biopsy testing is an effective way to identify SFN and its underlying causes in patients previously diagnosed with FM.

Methods: We studied 56 patients referred to peripheral nerve disease centers for evaluation of neuropathic pain who met the diagnostic criteria for FM. All underwent punch skin biopsy testing. If SFN was detected, patients underwent further laboratory testing to look for a potential cause for the neuropathy.

Results: Thirty-four of 56 patients (61%) had SFN, as indicated by reduced IENFD. Twenty-four of 34 patients with SFN (71%) had laboratory evidence that revealed an underlying etiology for the SFN.

Conclusions: More than half of the patients presenting with neuropathic pain and who met diagnostic criteria for FM had SFN detected by skin biopsy testing. A potential cause for neuropathy was identified in 71% of the patients with SFN. Skin biopsy testing to look for SFN is a very high-yield test in patients with FM who have neuropathic pain symptoms. Diagnosing SFN can facilitate the identification of potential causes for the neuropathy and alter their management.