Creutzfeldt-Jacob Disease: Two Case Reports

Creutzfeldt-Jakob Disease (CJD) is characterised by subacute progressive dementia, cerebellar ataxia, myoclonic jerks together with pyramidal and extrapyramidal signs. It is a rare prion disease and definitive diagnosis can only be made by biopsy. It becomes progressively worse and the death is the rule. We presented two CJD cases because of their demonstrative characteristics. A 43 year-old female and a 52 year-old male patient was suspected to be CJD due to presence of subacute severe cognitive deterioration, neuropsychiatric disturbances, myoclonic jerks, ataxia, pyramidal and extrapyramidal signs and also periodic spike and wave complexes in EEG. Patients were lost in a short period of time because of the complications of disease process. Medical autopsy were done in both cases for definitive diagnosis and autopsy results displayed characteristic pathologic findings of CJD. Patients were diagnosed as definitive sporadic CJD according to Master’s, French and European criterias. CJD should be considered in patients with rapidly progressive dementia, that starts with various neuropsychiatric symptoms. Although seen very rare, CJD is a untreatable, fatal disease. Therefore we emphasize that, preventive precaution should be taken when a CJD diagnosis is suspecte

Tolosa-Hunt Syndrome: A Report of Two Cases

Tolosa-Hunt syndrome (THS) is a painful ophthalmoplegia, characterized by cryptogenic granulomatous inflammation of the cavernous sinus and/or superior orbital fissure. Glucocorticoid treatment is used for both diagnostic and therapeutic purposes. According to the Headache classification subcommittee of international headache society criteria, magnetic resonance imaging (MRI) or biopsy is necessary for demonstration of the granulomatous inflammation. Here, we present two cases of THS with clinical and MRI findings

Ortner sendromu' nun nadir bir nedeni: Behçet hastalığına sekonder gelişen dev pulmoner arter anevrizması

Behçet Hastalığı nedeni bilinmeyen sistemik bir otoimmün vaskülittir. Hem arterleri hem de venleri tutarak ciddi sorunlara neden olabilir. Kalp ve intratorasik büyük damarların patolojilerine bağlı olarak sol rekürren laringeal sinirin kompresyonu sonucu gelişen ses kısıklığı Ortner Sendromu olarak ifade edilir. Ortner Sendromu' nun en sık nedeni mitral darlığa bağlı olarak sol atriyum genişlemesidir. Çeşitli intratorasik patolojiler bu tablonun nedeni olabilir. Bununla birlikte Behçet Hastalığı sonucu gelişen pulmoner arter anevrizmasına bağlı Ortner Sendromu nispeten nadirdir. Biz burada ses kısıklığı ile başvuran ve yapılan incelemeler sonucunda Behçet Hastalığı' na bağlı dev pulmoner arter anevrizması sonucu gelişen Ortner Sendromu olarak tanı alan 78 yaşında bir bayan hastayı sunuyoruzBehçet' s disease is a systemic autoimmune vasculitis of unknown etiology. It causes serious disability by affecting both arteries and veins. Hoarseness due to compression of the left recurrent laringeus nerve resulting from pathologies of the heart and intrathoracic great vessels is defined as Ortner's syndrome. The most common cause of Ortner's syndrome is left atrial enlargement due to mitral stenosis. Various intrathoracic pathologies may also be the reason. Beside, Ortner's syndrome due to primary pulmonary artery aneurysm as a feature of Behçet's disease is relatively rare. Herein, we report a case of a 78 year old female patient presenting with hoarseness and diagnosed as Ortner's syndrome resulting from a giant pulmonary artery aneurysm secondary to Behçet' s disease. J Clin Exp Invest 2015; 6 (1): 69-7

Leucine-Rich Glioma-Inactivated Protein 1 Antibody-Positive Polyradiculopathy Associated with Epstein-Barr Virus Infection

Epstein-Barr virus (EBV) has been associated with a plethora of neurological manifestations including polyneuropathy and polyradiculopathy. A 27-year-old man with a recent upper respiratory system infection presented with difficulty in walking. His neurological examination revealed reduced muscle strength in both proximal and distal lower limb muscles without sensory and autonomic signs. Needle electromyography showed abnormal spontaneous activity and reduced recruitment of motor units in muscles innervated by multiple lumbo-sacral roots. Cerebrospinal examination showed increased protein levels with normal cell counts. While spinal MRI was normal, whole-body CT and PET examination showed disseminated lymph node enlargement. Anti-EBV viral capsid antigen and anti-nuclear antigen IgG but not IgM was positive, whereas EBV PCR was negative in blood. Analysis of inguinal lymph node biopsy showed reactive lymphoid hyperplasia and EBV DNA. Leucine-rich glioma-inactivated protein 1 (LGI1) antibody was found in serum but not in CSF. All clinical, imaging, and electrophysiological findings improved following steroid and intravenous immunoglobulin treatment. These findings suggested the acute involvement of lumbo-sacral spinal roots and/or motor neurons. Purely motor polyradiculopathy has been reported in both EBV-positive and LGI1 antibody-positive patients, and EBV infection is known to precede different autoimmunemanifestations. Whether EBV infection may trigger LGI1 autoimmunity and cause involvement of spinal motor roots and/or motor neurons needs to be further studied

Electromyographic Findings in Overt Hypothyroidism and Subclinical Hypothyroidism

OBJECTIVE: Hypothyroidism may cause neurologic signs and symptoms as its effects neuromuscular system like many other systems. Subclinical hypothyroidism is the most common thyroid dysfuntion, it may cause neuromuscular signs and symptoms. In this retrospective study, it is aimed to compare neuromuscular symptoms and electromyographic (EMG) manifestations between hypothyroid patients and control group with normal thyroid function and without a disease causing polyneuropathy. METHODS: 31 overt hypothyroidic, 139 subclinic hypothyroidic patients and 50 individuals with normal thyroid function, without a disease causing polyneuropathy, as control group whom made EMG for another reason were included to the study. Neuromuscular symptoms, neurological examination and electrophysiological findings was obtained from the patient records. RESULTS: In our study, we observed frequent neuromuscular complaints such as fatigue, morning stiffness, cramp, general pain and paresthesia in favor of both for overt and subclinic hypothyroidism. Carpal Tunnel Syndrom(CTS), was statistically higher in overt hypothyroidism group than control group. CTS was also observed higher in subclinic hypothyroidism group when compared with control group but it didn't reach to statistical significance. We did not detect polyneuropathy in any group. Motor nerve velocity and compound muscle action potential amplitudes were found to be statistically significant difference between hypothyroid ve control group. CONCLUSION: Since motor fibres' and neuromuscular area's being affected in hypothyroidism, which we interpret to happen due to basal metabolism's slowing down, can show a significant recovery after thyroid replacement therapy. We consider that, in further studies, comparison of electrophysiological findings after treatment with the findings of pre -treatment is necessar