Gülden Akdal

Neuro-ophthalmology of invasive fungal sinusitis: 14 consecutive patients and a review of the literature

Invasive fungal sinusitis is a rare condition that usually occurs in immunocompromised patients and often presents as an orbital apex syndrome. It is frequently misdiagnosed on presentation and is almost always lethal without early treatment.

A case with cerebral thrombosis receiving tamoxifen treatment

Cerebral sinus thrombosis (CST) is known to be related to a number of underlying aetiologies including otitis media, trauma, pregnancy, birth control pills, tumours, malnutrition, dehydration, haematologic disorders and malignancy ( Fishman, 2000 ; Raizer and Abbott, 2000 ). We present the case of a patient with breast cancer receiving the antioestrogen drug tamoxifen who developed CST.

The prevalence of vestibular symptoms in migraine or tension-type headache

BACKGROUNDnWe assessed frequency of vestibular symptoms in Headache Clinic patients over 10 years.nnnMETHODSnA descriptive study of 5111 consecutive patients with tension-type headache or migraine, analyzed for dizziness/ vertigo accompanying headache and for a lifetime history of motion-sickness, cyclic vomiting, recurrent abdominal pain or atopy. Migraine patients were re-grouped as those with vestibular symptoms (dizziness/vertigo or motion sickness) and those without and their data then re-analyzed.nnnRESULTSnThere were 1880 migraine patients and 3231 tension-type headache patients. Significantly more migraine patients than tension-type headache patients experienced vestibular symptoms (p< 0.0001). The migraine with vestibular symptoms group was significantly younger (p< 0.05) had more aura, more phonophobia with migraine attacks (p< 0.0001). Menstruation and reported sleep problems impacted on headaches. While past history of cyclical vomiting, recurrent abdominal pain or atopy was about twice as common in migraine with aura and it was also more common in migraine with vestibular symptoms than migraine without vestibular symptoms.nnnCONCLUSIONSnVestibular symptoms are common in migraine patients. Migraine with vestibular symptoms might constitute a special group, one more likely to have had cyclic vomiting, recurrent abdominal pain or atopy.

Eye movement disorders in vitamin B12 deficiency: two new cases and a review of the literature.

Eye movement disorders are rarely reported in vitamin B12 deficiency. We describe two cases with eye movement disorder and vitamin B12 deficiency; one with bilateral internuclear ophthalmoplegia and the other with downbeat nystagmus. Both of the patients received replacement therapy but their eye movement disorders did not respond to treatment. We also review the nine previously reported cases.

MRI follow-up of basal ganglia involvement in subacute sclerosing panencephalitis

A 12-year-old male with subacute sclerosing panencephalitis is presented. Magnetic resonance imaging revealed basal ganglia involvement without white matter changes for several months. Basal ganglia changes are not infrequent in subacute sclerosing panencephalitis, but they tend to appear in advanced clinical stages. Prominent basal ganglia involvement may occur very rarely in subacute sclerosing panencephalitis. In our patient, serial magnetic resonance imaging demonstrated the involvement of white matter after 2 years of magnetic resonance imaging follow-up. In contrast with the neuroradiologic progression, our patients clinical status remained stable.

Is balance normal in migraineurs without history of vertigo

Objective.— To investigate by static posturography the occurrence of balance disorder in migraineurs without a history of vertigo during the interictal period.

Treatment responsive executive and behavioral dysfunction associated with Vitamin B12 deficiency

Vitamin B12 deficiency can manifest with various neuropsychiatric symptoms. We report a case of an individual with vitamin B12 deficiency presenting with personality, emotional and behavioral changes. Neuropsychological tests revealed disproportionate disturbance of executive functions. Single photon emission tomography (Tc99m HMPAO) showed right fronto-temporo-parietal hypoperfusion. After vitamin B12 injections, the patients executive and behavioral dysfunction disappeared and performance on neuropsychological tests was once again in the normal range.

Search for SCA2 blood RNA biomarkers highlights Ataxin-2 as strong modifier of the mitochondrial factor PINK1 levels.

Ataxin-2 (ATXN2) polyglutamine domain expansions of large size result in an autosomal dominantly inherited multi-system-atrophy of the nervous system named spinocerebellar ataxia type 2 (SCA2), while expansions of intermediate size act as polygenic risk factors for motor neuron disease (ALS and FTLD) and perhaps also for Levodopa-responsive Parkinsons disease (PD). In view of the established role of ATXN2 for RNA processing in periods of cell stress and the expression of ATXN2 in blood cells such as platelets, we investigated whether global deep RNA sequencing of whole blood from SCA2 patients identifies a molecular profile which might serve as diagnostic biomarker. The bioinformatic analysis of SCA2 blood global transcriptomics revealed various significant effects on RNA processing pathways, as well as the pathways of Huntingtons disease and PD where mitochondrial dysfunction is crucial. Notably, an induction of PINK1 and PARK7 expression was observed. Conversely, expression of Pink1 was severely decreased upon global transcriptome profiling of Atxn2-knockout mouse cerebellum and liver, in parallel to strong effects on Opa1 and Ghitm, which encode known mitochondrial dynamics regulators. These results were validated by quantitative PCR and immunoblots. Starvation stress of human SH-SY5Y neuroblastoma cells led to a transcriptional phasic induction of ATXN2 in parallel to PINK1, and the knockdown of one enhanced the expression of the other during stress response. These findings suggest that ATXN2 may modify the known PINK1 roles for mitochondrial quality control and autophagy during cell stress. Given that PINK1 is responsible for autosomal recessive juvenile PD, this genetic interaction provides a concept how the degeneration of nigrostriatal dopaminergic neurons and the Parkinson phenotype may be triggered by ATXN2 mutations.

Improvement in Tc-99m HMPAO brain SPECT findings during donepezil therapy in a patient with pure akinesia

A 58-year-old man presented with a history of disturbance in initiating gait. His history revealed meningoencephalitis five years prior to admission. Neurological examination included gait disturbance as difficulty in initiation and a hesitating speech with many freezing episodes and micrographia Magnetic resonance imaging (MRI) showed diffuse hyperintensity of frontal subcortical white matter on T2 weighted images. He was diagnosed with PA. l-Dopa up to the dosages of 1000 mg/ day and Selegiline 10 mg/day were given. First brain SPECT using technetium-99m labeled d,l-hexamethylpropylene amine oxime (Tc-99m HMPAO) was performed when he was taking l-dopa and Selegiline. In visual evaluation, hypoperfusion in bilateral frontoparietal cortex was seen (Fig. 2). Treatment with l-dopa and Selegiline produced no benefit. Donepezil 10 mg/day was begun. This therapy regimen resulted in dramatic clinical improvement within several days that was confirmed by blinded raters who watched the patient’s video recordings. During this response second brain perfusion SPECT study was repeated during donepezil therapy. Markedly increased perfusion in bilateral frontoparietal cortex was observed. This is the first case of PA to develop possibly after an episode of bacterial pneumococcal meningoencephalitis and who responded to donepezil as documented by changes in clinical findings and Tc-99m HMPAO brain SPECT studies.

Giant cell arteritis presenting with bilateral vertebral and axillary artery occlusions

A man aged 69 presented with a 1-month history of 3–4 brief, 1to 2-min episodes of imbalance daily. These attacks began after a 10-min attack of vertigo, nausea, dysarthria and perioral numbness. Four years previously he had had a 2-day attack of vertigo and vomiting, attributed by his general practitioner to food poisoning. He had also noted pulsatile tinnitus in the left ear but not headache, jaw pain, arm pain, visual problems, shoulder aches or constitutional symptoms. On examination, there were no neurological abnormalities. Systolic blood pressure in the right arm was approximately 70 mmHg by palpation, but could not bemeasured at all in the left; in the left leg it was 170/90, but therewere no pedal or popliteal pulses in the right. There was a left carotid bruit radiating to the orbit; the temporal arteries were normal to palpation. Brainmagnetic resonance imaging showed only a small, old, border-zone infarct in the left cerebellar hemisphere on T2 images. Brain magnetic resonance angiography showed bilateral vertebral artery occlusions and bilateral carotid siphon stenoses (Fig. 1a,b); the basilar artery filled, feebly, retrogradely through the posterior communicating arteries; the subclavian arteries were normal, but both axillary arteries were occluded (Fig. 1c) with prominent collateral circulation. The erythrocyte sedimentation rate was 97 mm/h. A temporal artery biopsy showed active giant cell arteritis. He was treated with an immediate left carotid siphon angioplasty to improve retrograde basilar blood flow through the posterior communicating artery, as well as coumadin and corticosteroids for 18 months. Although neither the vertebral nor the axillary arteries have recanalized, there was, immediately after the angioplasty, a marked and so far maintained, improvement in basilar artery perfusion and he remains asymptomatic from the vertebral and axillary artery occlusions 4 years after presentation. Although giant cell arteritis has a predilection for the external carotid artery and its branches, particularly the superficial temporal artery, causing headache, jaw claudication, scalp and tongue necrosis, and the posterior ciliary branches of the ophthalmic artery, causing ischaemic optic neuropathy, it can also affect other large muscular arteries, particularly the extracranial vertebral arteries, aswell as the internal carotid, coronary, subclavian, brachial and axillary arteries and the aorta itself, causing transient ischaemic attacks, stroke, myocardial infarction and upper limb