A Rare Coexistence of Paraneoplastic Cerebellar Degeneration: Papillary Thyroid Carcinoma.

Paraneoplastic cerebellar degeneration (PCD) is a rare neuroimmunological disease that may accompany tumors. In this article, we present a patient with progressive gait difficulty who was diagnosed with PCD and, in a rare comorbidity, with papillary thyroid carcinoma (PTC) following malignancy screening. A 46-year-old male patient reported having experienced poor balance for 2 years. A neurological examination revealed nystagmus, intention tremor, and ataxia, and anti-thyroid peroxidase and anti-thyroglobulin levels were found to be elevated. A brain MRI showed significant cerebellar atrophy in the superior vermis. Malignancy screening for PCD was performed, and thyroid ultrasonography revealed a nodule in the left lobe, while PET/CT detected elevated focal F-18 fluorodeoxyglucose uptake in the thyroid. Onconeuronal antibodies (anti-Hu, anti-Yo, anti-Ri, anti-amphiphysin, anti-Tr, anti-PPCA-2, anti-Ma, anti-CV-1, and anti-ANNA-3) were negative. Pathologic examination of the thyroid revealed PTC, for which the patient was treated with 0.4 g/kg intravenous immunoglobulin and referred to the medical oncology department. This case demonstrates that clinicians must be alert to the rare comorbidity of PCD and PTC.

An Unusual Recurrence of Miller Fisher Syndrome: Three Times in Eight Years.

Miller Fisher Syndrome (MFS) is an acute, autoimmune polyneuropathy usually associated with a good prognosis which is clinically characterized by ataxia, ophtalmoplegia, and areflexia. MFS has a monophasic course, double recurrence can be seen in rare cases. But three attacks are very rare. In this article, we discussed a rare case of MFS and approach to the treatment that recurred 3 times within a period of 8 years. It is emphasized that MFS cases may occur a recurring pattern, and plasma exchange should be definitely tried as a therapeutic approach when clinical deterioration occurs under intravenous immunoglobulin (IVIg) therapy.

Serum Prolidase Enzyme Activity Level: Not a Predictive Biomarker for Epilepsy.

OBJECTIVE: Oxidative stress (OS) and inflammation are considered responsible for the pathogenesis of epilepsy. Prolidase has an extremely important role in proline recycling for collagen synthesis. Higher than normal proline levels have been shown to increase OS. Furthermore, prolidase activity is associated with inflammation during fibrotic process. No study has yet investigated the relationship between epilepsy and prolidase enzyme activity (PEA). In this study, we aimed to contribute to the existing literature by assessing postictal PEA levels, which are correlated with inflammation and OS, to determine whether PEA levels may be used as a biomarker for epilepsy. MATERIALS AND METHODS: This study included patients with epilepsy who presented to the emergency department within first 6 h of a seizure. RESULTS: The epileptic group included 27 patients (16 males, 11 females) and the control group included 31 healthy individuals (11 males, 20 females). The mean age of the epilepsy (n=27) and healthy control group (n=31) was 43.1±20.2 and 51.9±21 years, respectively. Serum PEA levels were 1171.90±343.3 in the epileptic group and 1137.1±295.6 in the control group. There were no significant differences between two groups (p>0.05). CONCLUSION: Our study results suggest that although PEA is an enzyme associated with OS and inflammation, it is still not an ideal biomarker for epileptic patients. This study is important because it investigated PEA in patients with idiopathic epilepsy for the first time.

Atypical type of Hirayama disease: Onset of proximal upper extremity.

Hirayama disease is a rare, benign motor neuron disease. It has been proposed that the dura mater's posterior wall lacks sufficient elasticity in the lower cervical region and this causes the tense dura part to displace anteriorly upon flexion. The disease is described as involving unilateral upper extremity with a distal-onset. We reported weakness and atrophy of the proximal part of an extremity in a 45-year-old man who is diagnosed with Hirayama disease. Proximal onset is a rare type of Hirayama Disease. Clinicians must be alert of proximal involvement and the diagnosis should be confirmed with electrophysiological and flexion MRI studies.

Alteration of mean platelet volume in the pathogenesis of acute ischemic stroke: cause or consequence?

BACKGROUND AND PURPOSE: Platelets have a crucial role on vascular disease which are involved in pathogenesis of ischemic stroke. Platelet size is measured as mean platelet volume (MPV) and is a marker of platelet activity. Platelets contain more dense granules as the size increases and produce more serotonin and tromboglobulin (b-TG) than small platelets. In this study, the alteration of MPV values were investigated in patients with acute stroke, who had MPV values before stroke, during acute ischemic stroke and 7 days after the stroke. The relationship between this alteration and risk factors, etiology and localization of ischemic stroke were also investigated. METHODS: Sixty-seven patients with clinically and radiologically established diagnoses of ischemic stroke were enrolled into the study and stroke etiology was classified by modified Trial of Org 10 172 in Acute Stroke Treatment (TOAST) classification and, modified Bamford classification was used for localization and stroke risk factors were also evaluated. The platelet counts and MPV values from patient files in patients who had values before stroke (at examination for another diseases), within 24 hours of symptom onset and after 7 further days were analysed. RESULTS: MPV values increased after stroke (10.59±2.26) compared with acute stroke values (9.84±1.64) and the values before stroke (9.59±1.72) (p<0.0001); this alteration of MPV values occured 7 days after stroke (p<0.016). There was a positive correlation between age and MPV values during acute stroke (r=0.270; p<0.05). Patients with atrial fibrillation had higher alteration in the time of MPV compared with patients without atrial fibrillation (p>0.006). We assessed for gender, men (n=38) had a higher alteration in the time of MPV compared with women (n=29) (p=0.013). CONCLUSION: Although there was no alteration of platelet counts, MPV values were increased 7 days after stroke in patients with acute ischemic stroke.

The clinico-radiological spectrum of Dyke-Davidoff-Masson syndrome in adults.

Dyke-Davidoff-Masson syndrome (DDMS) is characterized by cerebral hemiatrophy, seizure, contralateral hemiplegia/hemiparesis, and mental retardation. In this study, clinical and radiological investigations of seven patients who were diagnosed with DDMS as adult age were evaluated and discussed. Seven patients (four male, three female) were included. The mean age ± SD of the patients was 46 ± 21 years. Clinical presentation of six patients was epileptic seizure. One patient was presented with head trauma due to a fall. Two patients had complex partial seizures, three patients had generalized tonic-clonic seizures (GTC), and one had GTC and myoclonic seizure. Mental retardation was in five patients. A congenital cause was detected in one patient in the etiologic investigation and acquired causes in two patients. In four patients, the etiology was not identified. We observed left-hemisphere involvement in four patients and right-hemisphere involvement in three patients. Brain imaging was performed by CT only in four patients and by MRI only in three patients. All patients were diagnosed with DDMS at adulthood. Atrophy in basal ganglia was detected in five patients, and atrophy in brain stem in four patients. Calvarial thickening was observed in four patients. Three patients had hyperpneumatization in mastoid cells. Sinus hyperpneumatization, including the paranasal and frontal sinuses, was seen in six patients. DDMS can also be diagnosed in adulthood symptomatically (mild-severe) or asymptomatically in adulthood. As a result, DDMS is a syndrome with wide clinical and radiological spectra that can be variably symptomatic at different stages of life.

Autoimmune neurological syndromes associated limbic encephalitis and paraneoplastic cerebellar degeneration.

INTRODUCTION: Autoimmune neurological syndrome is a group of disorders caused by cancer affecting nervous system by different immunological mechanisms. In this study, we aim to study the clinical symptoms, cerebrospinal fluid (CSF) findings, autoantibody tests, computed tomography (CT), magnetic resonance imaging (MRI) signs and treatment outcome of patients with autoimmune syndromes. METHODS: In this study, 7 patients (4 male, 3 female) diagnosed with autoimmune neurological syndrome were retrospectively examined. RESULTS: Five of patients were diagnosed with limbic encephalitis, two of them were paraneoplastic cerebellar degeneration. Confusion and seizure were the most seen symptoms. Two patients had psychiatric disturbances (28,5%) followed by seizure. Headache was seen in 2 patients (% 28,5), disartria in 1 patient (% 14,2), and gait disorder in 2 patients (28,5%). The duration of symptoms was 46 (3-150) days on average. CSF abnormalities were detected in 2 patients. CT and MRI of the brain was available in all patients. Five patients had involvement of mesiotemporal region, two patients had diffuse cerebellar atrophy. One of patients had anti-GABAR B1 positivity. Tumors were detected in 2 patients while investigation for paraneoplasia screening. CONCLUSION: Remission is only possible with the detection and treatment of the malignancy. Early diagnosis and treatment are of paramount importance.

Comparison of brain MRI angiography and brain MRI cisternography in patients with hemifacial spasm.

OBJECTIVE: The aim of this study was to compare the ability of brain magnetic resonance imaging angiography and brain magnetic resonance imaging cisternography of showing the neurovascular contiguity of the facial nerve in patients with hemifacial spasm. METHOD: This study included a total of 35 patients (28 females, 7 males), with an average age of 57.5 ± 13.8 years, who presented to our outpatient clinic of movement disorders. All patients were prospectively applied brain magnetic resonance imaging angiography and brain magnetic resonance imaging cisternography, and the results were evaluated by a radiologist who was blind to study protocol. RESULTS: The study included 35 patients, of whom 28 (80 %) were female and 7 (20 %) were male. Brain magnetic resonance imaging angiography demonstrated facial nerve compression of vascular origin in 5 (14.3 %) patients presenting with the clinical presentation of hemifacial spasm. Neurovascular compression of facial nerve was shown by brain magnetic resonance imaging cisternography in 13 (37.1 %) patients. CONCLUSION: No statistically significant difference was found between brain magnetic resonance imaging angiography and brain magnetic resonance imaging cisternography techniques in detecting a compressive interaction causing hemifacial spasm between facial nerve and adjacent vascular structures (p > 0.05).

Cerebral fat embolism diagnosed by cognitive disorder.

Fat embolism syndrome is a rarely seen complication of skeletal trauma, and it is seen at a rate of 2-5% after fractures of the long bones of the lower extremities. Its classic triad-+consists of hypoxemia, petechial bleedings on the skin and neurological findings. These neurological findings are highly variable and non-specific, and they can present with lethargy, irritability, delirium, stupor, convulsion or coma. In this report, a male case is presented who was diagnosed with cerebral embolism due to acute cognitive disorder after a segmental tibial fracture.