Effect of Liver Transplant on Neurologic Manifestations and Brain Magnetic Resonance Imaging Findings in Wilson Disease.

OBJECTIVES: Wilson disease is an autosomal recessive disorder of copper metabolism. It leads to copper accumulation in various organs (liver, eye, brain) and deteriorates their functions. Symptoms usually appear in the second and third decades of life. Neurologic symptoms and manifestations may appear 2 to 5 years after liver involvement, and neurologic symptoms are usually movement disorders. The main treatment objective is to decrease accumulation of copper by increasing urinary copper excretion. With early diagnosis and treatment, the quality of life of patients with Wilson disease evolves. In this study, we aimed to evaluate the effects of liver transplant on neurologic manifestations and radiologic findings in patients with Wilson disease. MATERIALS AND METHODS: Since 1988, our center has performed 642 liver transplant procedures. Fifty-three patients with Wilson disease received a liver transplant during this period, with 15 adults patients included in our study. All study patients were evaluated by the same neurologist and radiologist. Tremor was scored by the glass scale test. Radiologic evaluations were made by cranial magnetic resonance imaging. RESULTS: Before liver transplant, 4/15 study patients had tremor. In 1 patient, tremor was accompanied by dystonia; the patient's imaging findings and neurologic manifestations had regressed posttransplant. In the other 3 study patients with tremor, tremor decreased without any change in imaging findings. New-onset tremor was seen in 1 patient after liver transplant, but this patient had no observed imaging changes. This situation was correlated with immunosuppressive therapy. CONCLUSIONS: Neurologic recovery can be achieved in patients with Wilson disease with early diagnosis and treatment. Radiologic findings can be improved after therapy.

Ipilimumab treatment associated with myasthenic crises and unfavorable disease course.

Ipilimumab, a monoclonal antibody targeting the cytotoxic T lymphocyte antigen-4 is a treatment option for metastatic melanoma. This receptor serves as an inhibitor receptor on T lymphocytes and blockage of these results predisposition to autoimmune diseases. The patients with autoimmune disorders were excluded in clinical trials and there is no sufficient data of the treatment on these patients who already have an autoimmune disorder. Here, we report a case of myasthenia gravis who was treated with ipilimumab and we presented the long-term prognosis of the patient after treatment.

Epilepsy After Heart Transplant: A Single Center Experience.

OBJECTIVES: Cardiac transplant is the best treatment for patients with end-stage heart failure. Neurologic complications occur at a rate of 30% to 80% in patients undergoing cardiac transplant. Seizures occur at a rate of 2% to 20%. The main causative factors include immunosuppressant drug toxicity, infections, brain lesions, and metabolic disorders. Here, our aim was to determine seizure types and associated conditions in patients undergoing cardiac transplant and to report our treatment experience at our institution. MATERIALS AND METHODS: We retrospectively evaluated the medical records of 109 patients who underwent cardiac transplant between 2004 and 2016. We recorded demographic data, immunosuppressive treatment, seizure type, cause, recurrence rate, and treatment. RESULTS: Of 109 patients, 13 had seizures after cardiac transplant. Our study involved 69 adult and 40 pediatric patients. The pediatric patients had an age range of 1 to 17 years, with a mean age of 9.6 years (22 female and 18 male patients). Five pediatric patients had seizures (4 female and 1 male patient). The seizure causes included 2 postarrest hypoxic encephalopathies and 3 posterior reversible encephalopathies. Adult patients ranged from 18 to 63 years old, with a mean age of 42.3 years (54 male and 15 female patients). Eight patients in the adult patient group had seizures (5 female and 3 male patients). Seizure causes were ischemic cerebrovascular events in 2 patients, metabolic disorders in 2, posterior reversible encephalopathies in 3, and postarrest hypoxic brain in 1. CONCLUSIONS: Seizure is an important complication after cardiac transplant. At our institution, the most common cause of seizure was posterior reversible encephalopathy, with immunosuppressant drugs being responsible.

Seizure as a neurologic complication after liver transplant.

OBJECTIVES: Seizure is a common complication after liver transplant and has been reported to occur in up to 42% of patients in different case series. Multiple factors can trigger seizures, including immunosuppressive toxicity, sepsis, metabolic imbalance, and structural brain lesions. The aim of this retrospective study was to evaluate seizure types and associated factors in adult liver transplant patients. MATERIALS AND METHODS: We retrospectively evaluated the medical records of 142 adult patients who received a liver transplant between 2005 and 2013. We recorded demographic data, immunosuppressive treatment, seizure type, cause, recurrence, and treatment. RESULTS: Of the 146 patients, 23 (15.7%) had a seizure after the liver transplant. This group included 10 females and 13 males, with ages ranging between 18 and 63 (39.9 ± 14.8 y). Generalized tonic-clonic seizures were the most common, occurring in 20 patients (87%). We observed complex partial seizure and status epilepticus in 1 and 2 patients. Immunosuppressive drug-related seizure occurred in 8 patients (34.8%) with normal drug blood levels, and all but 1 of these patients experienced seizure within the first week after transplant. Multiple factors (26.1%), metabolic imbalance (17.4%), structural lesion (13%), and sepsis (8.7%) were the other factors identified as underlying conditions. CONCLUSIONS: In conclusion, seizure occurred in a significant proportion of patients who underwent liver transplant. Immunosuppressive drugs were the most common factor associated with seizure occurrence and drug cessation prevented seizure recurrence.

Neurologic complications after liver transplant: experience at a single center.

OBJECTIVES: Neurologic complications occur frequently after liver transplants. Up to 43% of patients experience severe postsurgical neurologic complications. These complications are significantly associated with longer hospital stay, morbidity, and mortality. The aim of this retrospective study was to evaluate the type and incidence of neurologic complications after liver transplants in adult patients. MATERIALS AND METHODS: We retrospectively evaluated the medical records of 176 adult patients who had undergone liver transplants between 1995 and 2013. We recorded the demographic data, type of neurologic complications, type, and level of immunosuppressive treatment, and cause of liver failure. RESULTS: Our study sample consisted of 48 deceased-donor liver transplants and 128 living-donor transplants (n = 176). Fifty-three of the patients (30.1%) were female. The age range of the total sample was 18 to 66 years (mean age, 43.1 ± 13.7 y). As immunosuppressive treatment, most patients received tacrolimus alone (52%) or tacrolimus combined with mycophenolate mofetil (33%). Neurologic complications occurred in 74 of the patients (42%). The most common neurologic complications were diffuse encephalopathy (22.2%) and seizure (14.2%). Other neurologic complications were posterior reversible encephalopathy (1.7%), peripheral neuropathy (1.7%), cerebrovascular disease (1.1%), and central nervous system infection (1.1%). Age, cause of liver failure, and type of transplant were not associated with occurrence of neurologic complications. CONCLUSIONS: There was a high incidence of neurologic complications after liver transplants. Diffuse encephalopathy and seizure were common complications. Physicians should be aware of the high risk of neurologic complications after liver transplants. Factors such as immunosuppressive toxicity and metabolic imbalance that predispose patients to neurologic complications after liver transplants should be evaluated immediately, and treatment of postoperative neurologic complications should be initiated as early as possible.

Spontaneous intra-cerebral hemorrhage: A retrospective study of risk factors and outcome in a Turkish population.

BACKGROUND AND PURPOSE: Stroke, which remains the third leading cause of death after heart disease and cancer in developed countries, is a disorder causing permanent neurologic disability. Even though, hemorrhagic strokes are seen less than the ischemic type, they are more fatal. We studied the risk factors for spontaneous intra-cerebral hemorrhage (ICH) to direct the proper preventive treatment modalities and the effects of these factors on mortality as well as applied therapeutic strategies on survival. MATERIALS AND METHODS: The archive records of 106 patients (60 male, 46 female) who were diagnosed with spontaneous ICH in Baskent University Hospital, Ankara, between January 2003 and September 2008, were assessed retrospectively. RESULTS: The mean age was found as 62.5. The most frequent risk factor was hypertension (73.5%); 69.2% of these hypertensive patients had uncontrolled blood pressure levels. The mortality rate was detected as 34.9% and patients were found to die approximately within 9 days after ICH. Older age, increased hemorrhage volume, ventricular extension of hemorrhage, and the presence of midline shift were found to significantly correlate with increased mortality (P < 0.05). Patients who underwent surgical therapy showed a longer survival rate (P = 0.016); however, no association was found between medical and surgical therapy in terms of mortality (P = 0.555). CONCLUSION: The results of this study suggest that effective control of blood pressure is important in the prevention of spontaneous ICH; clinical and radiological findings with treatment modalities influencing mortality should be carefully managed.

Angiotensin I-converting enzyme gene (I/D) polymorphism in patients with migraine.

In addition to the wide expression in many tissues including vascular endothelial cells, production of angiotensin II and degradation of bradykinin may indicate that angiotensin-converting enzyme could be involved in vascular tension and blood pressure. It has been reported that the deletion allele of the angiotensin-converting enzyme gene is associated with increased serum angiotensin-converting enzyme levels and linked to cerebrovascular diseases. In this study, the possible association of migraine with aura with the angiotensin-converting enzyme deletion-deletion (DD) and the angiotensin-converting enzyme insertion-deletion (ID) genotype was investigated in Turkish patients. To investigate the role of the angiotensin-converting enzyme I/D polymorphism in Turkish patients with migraine with aura, we analyzed the I/D genotype of 53 patients with that disorder. Twenty-two control subjects, who are volunteer Turkish patients without migraine, were included in the study. The frequency of the angiotensin-converting enzyme D/D genotype was statistically significant more frequent in patients with migraine with aura (81.1%) than in controls (59.1%) (P < .05). No differences were found regarding the I/I genotype and the I/D genotype between the 2 groups (P > .05). The results of our study revealed that the angiotensin-converting enzyme D/D genotype was more frequent in patients with migraine with aura than in controls. This might suggest that the angiotensin-converting enzyme D/D genotype may be a genetic risk factor for migraine with aura in Turkish patients.

Cardiac rupture.

BACKGROUND: Cardiac rupture is an unusual cause of chest pain and sudden cardiovascular collapse. This diagnosis may be easily forgotten while managing a patient in extremis in the initial minutes of evaluation and resuscitation in the emergency department (ED). OBJECTIVES: To report the benefit of immediate bedside cardiac ultrasonography in the diagnosis of cardiac rupture and its influence on emergent intervention in the ED. CASE REPORT: The initial electrocardiogram, performed within 5 min of arrival, of a 65-year old man who presented with 20 min duration of chest pain, showed a biphasic T wave in V1 and inverted T wave in V2, without ST-segment elevation myocardial infarction. Fifteen minutes later, he lost consciousness and was pulseless without a shockable rhythm on the monitor. Chest compressions were started and the patient was intubated. Echocardiography was performed at the bedside by the emergency physician. Cardiac contractility was grossly decreased in both ventricles and a large amount of pericardial fluid was seen. Two attempts at ultrasound-guided pericardiocentesis yielded only a few milliliters of blood. Interruptions in chest compressions were minimized during pericardiocentesis. Before transport of the patient to the operating room for definitive repair, asystole occurred. On the subsequent echocardiogram, heart contractions were absent and a hematoma was seen in the pericardial space. Resuscitation efforts were stopped. An autopsy was not performed per family request. CONCLUSIONS: Typical ultrasonographic findings of cardiac rupture were present in this patient, who presented in extremis with chest pain. Early bedside echocardiography can be helpful in directing the initial care of critically ill patients.

Bilateral facial nerve enhancement demonstrated by magnetic resonance imaging in Guillain-Barré syndrome.

Guillain-Barré syndrome (GBS) is an acute inflammatory demyelinating peripheral nerve disorder. It is known that gadolinium enhancement on magnetic resonance imaging (MRI) reflects alteration of the blood-nerve barrier secondary to inflammation. Enhancement of the cauda equina roots with gadolinium on lumbosacral magnetic resonance imaging have been demonstrated in several reports. Although about 50% of GBS patients clinically exhibit facial nerve involvement, it has never been demonstrated on MRI. We aimed to observe facial nerve involvement in a GBS patient who has prominent facial diplegia. With the guidance of the literature, we predict that MRI in selected GBS patients may be an adjunct tool for the clinician in both diagnosis and monitoring the treatment response.

Neurologic complications after renal transplant.

OBJECTIVES: Neurologic complications are a significant cause of morbidity and mortality in patients who undergo transplants. We sought to evaluate the nature and incidence of neurologic complications in patients undergoing a renal transplant. PATIENTS AND METHODS: Between January 2005 and December 2007, 132 adults (35 women, 97 men; mean age, 34.32 -/+ 0.90 years) underwent a renal transplant at our institution. Associated comorbid medical conditions, presenting neurologic symptoms, and type of immunosuppression were obtained from patients' medical records. RESULTS: Major indications for renal transplant were hypertensive nephropathy (14.4%), vesicoureteral reflux (11.4%), and idiopathic causes (21.2%). Mean follow-up was 17.26 -/+ 0.89 months (range, 2 weeks to 40 months). Twenty neurologic complications were found in 18 patients (6 women, 12 men; mean age, 33.83 -/+ 2.37 years). Presenting symptoms included posterior leukoencephalopathy syndrome, 1 (5.6%); cephalgia, 10 (55.6%); cerebral infarcts, 2 (11.1%); seizure, 3 (16.7%); tremor, 2 (11.1%); encephalopathy, 1 (5.6%); and sinus thrombosis, 1 (5.6%). Immunosuppressive agents were the primary cause of 16 of the 20 neurologic complications. Effectiveness and complications of cyclosporinewere screened for a total of 1858.50 months, tacrolimus for 853.50 months, and sirolimus for 620 months; 50.2% of the neurologic complications appeared during the first 3 months after transplant; the blood level of immunosuppressive medications did not need to be higher than normal in every case. DISCUSSION: In addition to cyclosporine and tacrolimus, we suggest (for the first time) sirolimus as a cause of neurocomplications after renal transplant.

A diagnostic challenge of Parkinson's disease: intractable hiccups.

Parkinson's disease is a common neurodegenerative disorder and the diagnosis depends mostly on clinical assessment so it is important to be aware of less common symptoms of the disease for correct diagnosis and therapy. We describe a patient with intractable hiccups as a very unusual presentation of Parkinson's disease.

Abnormal diffusion-weighted imaging findings in an adult patient with acute cerebellitis presenting with a normal magnetic resonance imaging.

Acute cerebellitis is an unusual central nervous system complication of infectious disease often due to viral etiology. Diagnosis is aided by neuroimaging studies, actually by magnetic resonance imaging in the first place, which shows increased signal intensity on T2-weighted images. However, conventional magnetic resonance imaging may be unrevealing in some of the cases, and additional workup such as single photon emission computed tomography and diffusion-weighted imaging may be required. We present a case of acute cerebellitis in a 35-year-old woman diagnosed by diffusion-weighted imaging.

Treatment differences in cases with venous angioma.

Venous angiomas were found to be the most common cerebral vascular malformations, composing 63% of such lesions in two autopsy series. Annual bleeding risk associated with venous angiomas is about 0.22 % per year. Venous angiomas are generally silent lesions because of their dynamic features, and are low flow and low pressure vascular structures draining normal brain tissue. An angioma rarely causes symptoms such as bleeding, seizure, hemifacial spasm, trigeminal neuralgia, aqueduct compression, nonhemorrhagic infarction and thrombosis of the draining vein. Even if it should bleed, the lesion can be managed conservatively in asymptomatic or mildly symptomatic patients. In this paper we report two venous angioma cases. The first patient bled twice in a short period of time and the angioma was located at the posterior fossa next to the left lateral recess. The second patient recently suffered a cerebral stroke that was located in the vicinity of the right caudate nucleus and not associated with the venous angioma that was located next to the left caudate nucleus. This patient had been under warfarin sodium treatment for 14 years due to his previous coronary artery bypass surgery, but unknowingly there was a venous angioma located next to the caudate nucleus.

Subclinical hypothyroidism, hyperhomocysteinemia and dyslipidemia: investigating links with ischemic stroke in Turkish patients.

OBJECTIVES: Hyperhomocysteinemia is a risk factor for ischemic stroke. Hypothyroidism may cause hyperhomocysteinemia. To date, no works have examined the association between hypothyroidism and hyperhomocysteinemia in ischemic stroke. We aimed to investigate the roles of hypothyroidism and hyperhomocysteinemia in ischemic stroke, and whether any relationship exists between hypothyroidism and hyperhomocysteinemia in ischemic stroke patients. METHODS: The study included 249 ischemic stroke patients and 102 patients with no history of stroke. Patients were evaluated for conventional risk factors and levels of homocysteine, thyroid-stimulating hormone, vitamin B12 and folic acid. RESULTS: Ten (4%) patients in the ischemic stroke group had subclinical hypothyroidism. We did not find any overt or subclinical hypothyroidism in the control group. Hypothyroidism was higher to a statistically significant degree in the ischemic stroke group (p<0.05). Both hyperhomocysteinemia and hypothyroidism were associated with ischemic stroke patients. However, no association was found between hyperhomocysteinemia and hypothyroidism. Ischemic stroke patients with hypothyroidism had lower levels of HDL cholesterol and levels of total cholesterol/HDL-C and LDL-C/HDL-C were higher than those of ischemic stroke patients without hypothyroidism. DISCUSSION: Hypothyroidism is associated with ischemic stroke. Low HDL cholesterol, high total cholesterol/HDL-C and high LDL-C/HDL-C were associated in ischemic stroke patients with hypothyroidism. Hyperhomocysteinemia was not found to be associated with ischemic stroke patients with hypothyroidism.

Is low blood magnesium level associated with hemodialysis headache?

OBJECTIVE: The aim of this study was to evaluate the prevalence, demographic, clinical features, and possible risk factors for hemodialysis headache (HDH). BACKGROUND: HDH has been recognized for many years, but the pathophysiology of this condition is not known. High arterial blood pressure, decreased serum osmolality, sodium washout, and high blood urea nitrogen level are reported risk factors for HDH. Low serum magnesium (Mg) level is known to cause some types of headache, including migraine (menstrual migraine in particular), tension-type headaches, and cluster and posttraumatic headaches. Low Mg has also been reported in HDH patients. METHODS: A total of 250 hemodialysis (HD) patients were questioned about problems with headache. Of these, 75 were diagnosed with HDH according to the revised International Headache Society criteria for 2003. Eighty age- and sex-matched HD patients without HDH were selected as a control group. For each HDH and control subject, arterial diastolic and systolic blood pressure, body weight, and serum levels of sodium, blood urea nitrogen, creatinine, and Mg were measured before and after one HD session. Urea reduction rate and ultrafiltration were determined. Serum levels of phosphorus, calcium, albumin, and parathormone were measured only before the session. Findings in the HDH and control group were statistically compared. RESULTS: As noted, 75 (30%) of the total 250 HD patients surveyed were diagnosed with HDH. The mean headache duration in this group was 5.17 +/- 5 hours. Vertex location, bilateral headache, dull nature, and moderate severity were the most prevalent features of HDH. There were no statistically significant differences between the HDH and control groups with respect to causes of end-stage renal disease. There were no significant differences between the HDH and control groups with respect to predialysis values for blood urea nitrogen, body weight, and arterial blood pressure (P > .05), and the same was true for comparisons of the postdialysis values for these parameters. The mean predialysis sodium level in the HDH group was higher than in the control group (P= .003). Both the mean predialysis and mean postdialysis Mg levels in the HDH group were significantly lower than the corresponding levels in the control group (P= .05 and P= .02, respectively). CONCLUSIONS: The results suggest that low blood Mg level and high blood sodium level may be risk factors for HDH. Magnesium supplementation may help patients with HDH whose serum Mg levels are found to be low.

Silent cerebral infarct in patients with mitral valve prolapse.

It is still not clear whether mitral valve prolapse (MVP) is a risk factor for ischemic stroke. The aim of this study was to evaluate whether uncomplicated MVP is a risk factor for silent cerebral ischemic events. Fifty-two patients with uncomplicated MVP and 46 control subjects without MVP were included in the study. All subjects were evaluated for silent cerebral infarct (SCI) with a magnetic resonance imaging. Five (9.6%) of the patients who had MVP but no other risk factors for ischemic cerebral events had SCI. The results suggest that uncomplicated MVP is a risk factor for SCI, and that patients with MVP should receive anti-platelet-aggregating drugs.

Personality, depression, and anxiety as risk factors for chronic migraine.

This article evaluated risk factors such as personality, depression, and anxiety in relation to migraine transformation in Turkish patients. Thirty-seven patients with chronic migraine and 50 patients with episodic migraine were investigated The scores for hysteria, hypochondriasis, psychasthenia, depression, and social introversion were all significantly higher in the chronic-migraine group than the episodic-migraine group. Seventy-four percent of the chronic-migraine group and 26% of the episodic-migraine group had depression. Eighty percent of the chronic-migraine group and 36% of the episodic-migraine group had anxiety. The results suggest that depression, anxiety, and personality characteristics such as hysteria, hypochondriasis, psychasthenia, depression, and social introversion may be associated with chronic migraine in Turkish patients.