Initial Presenting Symptom Research Articles

Clinical and Histopathological Study of Struma Ovarii: A Rare Ovarian Tumor

Struma ovarii or monodermal teratoma is a specialized ovarian neoplasm which mainly constitutes mature thyroid tissue. It is a rare tumor which comprises 1% of all ovarian tumors and 2.7% of all dermoid tumors. Thyroid tissue can be observed in 5-15% of dermoid tumors but to designate the tumor as struma ovarii, it must comprise more than 50% of the ovarian tissue. This study was conducted in the Department of Pathology, Manipal Teaching Hospital in Pokhara, Nepal over a period of 10 years (Jan 2006 to Sep 2015). Age, clinical findings, pre- operative imaging diagnosis, size and side of the tumor, gross and microscopic findings along with type of surgery performed are included in the study. During this 10 years period, there were 7 cases of struma ovarii with age ranging from 26 to 56 years. 2 cases had tumor on the right and 4 cases had tumor on the left side while 1 case had bilateral struma ovarii. Initial presenting symptom was palpable mass, abdominal pain and vaginal bleeding. The size of the tumor ranged from 4 to 15 cm. The capsule was smooth and cut surface shows multiloculated cyst filled with greenish to pale brown gelatinous thick fluid. Microscopic examination revealed well encapsulated tumor composed entirely of thyroid follicles. Diagnoses of struma ovarii were made in all cases. The preoperative imaging may not exactly give the diagnosis. Clinically, lesser age group was more affected and left side is more commonly involved in our series, in contrary to other literature. Out of 7 cases, bilateral struma ovarii was seen in 1 patient. No malignant features were seen in any of these cases.

Proximal arm weakness is the most common presentation in young Korean soldiers diagnosed as having hereditary neuropathy with liability to pressure palsy (HNPP).

Because patients with hereditary neuropathy with liability to pressure palsy (HNPP) are diagnosed mostly in their 20s, they are likely to experience their first major neurological symptoms during military training. We aimed to analyse the clinical characteristics and electrodiagnostic study findings of patients diagnosed with HNPP during their military service. We identified patients diagnosed as having HNPP in 2011-2014 and investigated their initial symptom presentation, the location and severity of their weakness, the causative event, the results of electrodiagnostic studies and the results of the genetic analysis of the PMP-22 gene. Among the 36 patients included in the study, 19 (52.8%) patients had upper extremity paraesthesia with proximal arm weakness caused by brachial plexus lesion. Distal upper extremity symptoms were found in 12 (33.3%) patients, and leg paraesthesia was present only in five (13.9%) patients. Among the 19 patients who had proximal arm weakness, the most common cause of weakness was the performance of push-ups as a punishment (36.8%), and strenuous push-up exercise was the second cause of this symptom (21.1%). Brachial plexus lesion leading to proximal arm weakness and paraesthesia was the most common presentation in soldiers with HNPP, and strenuous push-up activity was the major leading causative event in this condition.

Diabetic Ketoacidosis as the Initial Presenting Symptom of Pancreatic Adenocarcinoma

Diabetic Ketoacidosis as the Initial Presenting Symptom of Pancreatic Adenocarcinoma

Psychiatric consultations and the management of associated comorbid medical conditions in a regional referral hospital

Background . Psychiatrists are often called upon to evaluate patients with a medical condition and psychiatric symptoms, either as a complication thereof or initial presenting symptoms. There are often grey areas with regard to neuropsychiatric disorders in which psychiatrists and specialists from other clinical disciplines would need to co-manage or share ideas on the comprehensive treatment of a presenting patient. Objectives. This study was undertaken to provide a demographic and clinical profile of all patients consulted by the consultation-liaison psychiatry (CLP) service at the Helen Joseph Hospital (HJH) in Johannesburg, and to describe the clinical management of patients admitted with a diagnosis of a mental disorder associated with a comorbid medical condition, including delirium, dementia and a mood or psychotic disorder due to a general medical condition. Methods. A retrospective record review of all patients referred to the HJH CLP team over a 6-month period. Results. A total of 884 routine and emergency consultations were done for 662 patients (males n=305; females n=357) between the ages of 13 and 90 years who were referred from various other clinical departments. The most common documented reason for referral was a request for assessment (n=182; 27.5%), which consisted of mental state assessment, reconsultation and assessing capacity. A total of 63 patients (10.0% of cases consulted) were admitted to either the medical or psychiatric wards with a confirmed diagnosis of delirium, dementia and/or a mood or psychotic disorder due to a general medical condition (although admission wards were identified in 55 files only). The medical wards admitted the majority (n=37; 67.3%) mostly for delirium (n=28; 50.9%). HIV was identified as the most common systemic aetiological factor (n=23; 67.7%). Conclusion. In this study, a female patient between 31 and 45 years of age was slightly more likely to be referred to the HJH CLP service for assessment, and was more likely to be managed in the medical wards for delirium, which was most often associated with HIV/AIDS. The study highlighted the need for development of guidelines to facilitate adequate and effective use of this service for the local practice of CLP in a general specialist referral hospital like HJH, which would cover the following: clinical management; training needs; and administrative procedures.

Clinical profile and outcome of neonates admitted to the Neonatal Care Unit at a teaching hospital in Lalitpur, Nepal

Background: Neonatal period is a vulnerable time in which the newborn has to adapt to a totally new environment and is susceptible to many problems, which may even be life threatening. It is found that neonatal mortality rate is decreasing in Nepal but at a slower pace than infant and child mortality. In order to improve neonatal outcome, it is crucial to identify the areas where health care can be improved.Objectives: This study was conducted to analyze the spectrum of diseases and outcomes at a level two NICU at a tertiary care teaching hospital.Methods: A descriptive observational study conducted at the neonatal care unit in the Department of Paediatrics at KIST Medical College, Lalitpur, Nepal over one year from 14th of April 2013 to 13th April 2014. Data on age at admission, gender, gestational age, birth weight, initial presenting symptoms at admission, fi nal diagnosis and outcome were collected from in patient records at the hospital record section and analyzed using SPSS 17.0.Results: A total of 279 neonates were studied. The commonest causes of admission were infection (45.9%), followed by respiratory conditions (14.7%), neonatal jaundice (12.5%), perinatal asphyxia (9.3%) and prematurity/ small for gestational age (SGA) (6.5 %). Most of the admissions (62%) were during the fi rst 24 hours of life. The majorities, 89.2%, were term gestation and 10.8% were preterm. Most of the preterm babies were late preterms with gestational ages between 35- 36 weeks (73% of preterms were late preterms). Total of four mortalities (1.4%) occurred, three of which were due to perinatal asphyxia and one was a Down’s syndrome with overwhelming sepsis.Conclusion: In this study, infections followed by respiratory conditions were the leading causes of admission. Most of the admissions occurred during the fi rst 24 hours of life. This emphasizes the fact that attentiveness during the fi rst day of life, simple measure to prevent infection and early detection and treatment may go a long way in improving neonatal outcome.Journal of Kathmandu Medical CollegeVol. 3, No. 4, 2014Page: 144-148

Acute Psychosis in an Adolescent Treated With Infliximab for Crohn's Disease.

To the Editor: Infliximab is a monoclonal antibody against tumor necrosis factor-α (TNF-α) and has been shown to be 81% more efficacious than placebo in Crohn’s disease patients who have had an inadequate response to conventional therapy.1 While there are case reports of suicide attempts after treatment with infliximab for inflammatory bowel diseases,2,3 and reports of anti–TNF-α treatment leading to psychosis in elderly patients with rheumatologic disorders,4 to our knowledge, this is the first case of a pediatric patient with Crohn’s disease who developed acute psychosis after treatment with infliximab. Case report. A 15-year-old white male high school honors student, with a history of Crohn’s disease since age 12 years and a history of obsessive-compulsive disorder and attention-deficit disorder, received his first 3 infliximab infusions over a 2-month period for treatment of poorly controlled Crohn’s disease. One month after the third infusion, his teacher and parents noticed odd changes in his personality, which included inappropriate verbal outbursts, increased agitation, and defiant behavior over the course of 2 days. He then became verbally threatening and combative with his mother and was brought to the emergency department. In the emergency department, he was seen by psychiatry staff and found to have disorganized thought process with loose associations, echolalia, delusions of thought broadcasting and grandeur, distractibility, and severe psychomotor agitation. He had no history of recreational drug or alcohol use. Extensive workup results were all within normal limits including a head computed tomography scan, brain 3 magnetic resonance imaging, routine electroencephalograph, Lyme Western blot, urinalysis, lumbar puncture, cerebrospinal fluid culture, serum herpes simplex virus polymerase chain reaction, rapid plasma reagin, vitamin B12, folate, thyroid-stimulating hormone, stool culture, stool ova and parasite, and urine toxicology screen. The patient was started on haloperidol, which was titrated to 1 mg twice a day. Although this tempered his agitation and aggression, his psychosis did not completely resolve, and he experienced an acute dystonic reaction that led to the discontinuation of the medication. He was discharged home but came back in a few days for worsening behavior along with agitation and abusive speech. Treatment with olanzapine titrated to 7.5 mg twice a day resulted in resolution of his psychotic symptoms. However, on the fourth day of olanzapine treatment, a fourth infliximab infusion was administered, and shortly after, he suddenly became agitated and combative and appeared to experience visual hallucinations. The infliximab treatment was terminated, and he was discharged home with outpatient follow-up. At present, olanzapine has also been discontinued. He attends weekly group therapy at an outpatient facility and has returned to his normal mental status. Given the timing of his initial presenting symptoms within the 3 months of his initial infliximab infusions and the immediate relapse of psychotic symptoms after his fourth infliximab infusion, we believe that the patient’s psychosis is best explained by a psychotic reaction to infliximab. He was consistently psychotic with no fluctuations of consciousness to suggest coincidental delirium, and no substance-induced cause was identified. It is interesting to note that his psychosis responded better to an atypical neuroleptic compared to a typical one. There is evidence that atypical neuroleptics inhibit activated microglia release of proinflammatory cytokines, especially nitric oxide and TNF-α.5 Anti–TNF-α therapy has numerous well-recognized adverse events, such as infection and malignancy. However, there is very little information in the literature pertaining to psychiatric side effects. We suggest that patients treated with infliximab for Crohn’s disease could experience an acute psychosis. Treatment with an atypical antipsychotic may be considered over the typical antipsychotics. However, more controlled prospective studies are required to confirm our suspicion of causality between infliximab and psychosis to guide treatment.

P-063 Clinical outcomes in patients with gastric cancer bleeding as initial presenting symptom

P-063 Clinical outcomes in patients with gastric cancer bleeding as initial presenting symptom

The clinical characteristics and early detection of postpartum choriocarcinoma.

This study aimed to identify the clinical and demographic characteristics and prognosis of patients with conditions diagnosed with postpartum choriocarcinoma based on the International Federation of Gynecology and Obstetrics 2000 prognosis scoring system or based on pathologically confirmed choriocarcinoma and to analyze the patients' clinical symptoms for early detection of this disease. Between January 1983 and August 2013, 24 consecutive women with postpartum choriocarcinoma were treated at 2 hospitals. Data on clinical and demographic characteristics, including initial presenting symptoms, type of antecedent pregnancy, fetal complications, and prognosis of these patients, were analyzed. According to the time interval between the previous delivery and the onset of disease, patients were divided into 2 groups: the short and long interval groups. The most common symptom among the 24 patients with postpartum choriocarcinoma was irregular vaginal bleeding (14/24); in some cases, bleeding was caused by metastatic foci (7/24). Massive genital bleeding causing emergency hysterectomy and several obstetric complications, such as unknown severe fetal anemia and fetal growth retardation, was only observed in the short interval group. The overall primary remission rate was 91.7%. The most common symptom of patients with postpartum choriocarcinoma in the short and long interval groups was genital bleeding, and the overall prognosis may be improved by introduction of an appropriate chemotherapy regimen. Careful pathological examination of the placenta is needed in cases of fetomaternal hemorrhage, unknown fetal anemia, and abnormal obstetric events, including premature delivery, still birth, and infantile growth retardation, for the early detection of intraplacental choriocarcinoma.

Clinical features, outcomes, and genetic analysis in Korean children with Alagille syndrome.

Alagille syndrome (AGS) is a multisystem autosomal dominant disorder that affects the liver, heart, eyes, face, bone, and other organs. AGS is caused by mutations in one of two genes, JAG1 or NOTCH2. We evaluated clinical features, outcomes, and the presence of JAG1 and NOTCH2 mutations in Korean children with AGS. Between January 1997 and December 2013, 19 children were diagnosed with AGS at Asan Medical Center, Seoul, Korea. Their clinical features, outcomes, and JAG1 and NOTCH2 mutation status were retrospectively analyzed. The prevalence of clinical features in the 19 patients was as follows: dysmorphic facial features, 100% (n = 19); liver symptoms, 89% (n = 17); cardiac symptoms, 95% (n = 18); ophthalmologic symptoms, 67% (n = 10); skeletal deformities, 47% (n = 9); and renal symptoms, 21% (n = 4). JAG1 mutations were identified in 14 patients. The 13 different JAG1 mutations, seven of which were novel, included four deletions, three insertions, two missense mutations, three nonsense mutations, and one indel mutation. No NOTCH2 mutations were found. Two patients who received liver transplantation due to liver failure were still alive. Two patients died of comorbidities related to AGS: one of cardiac failure and one of hepatic failure. This study describes the clinical characteristics of 19 Korean AGS patients with seven novel JAG1 mutations. Neonatal cholestatic jaundice was the most common initial presenting symptom; thus the presence of neonatal cholestasis warrants screening for syndromic features of AGS. Complex heart anomalies and progressive liver dysfunction resulted in significant morbidity and mortality in AGS.

P89 – 2878: Neuronal ceroid lipofuscinosis-2 (CLN2) natural history and path to diagnosis: International experts' current experience and recommendations on CLN2 disease, a type of Batten disease, resulting from TPP1 enzyme deficiency

Objective Describe current practices and challenges related to diagnosis and natural history of CLN2 disease. Methods 18 international neuronal ceroid lipofuscinosis experts (clinicians, academic researchers, and diagnostic laboratory directors) answered a comprehensive survey on CLN2 disease. Topics included classification, natural history and path to diagnosis. Results Although a diagnostic algorithm exists, clinical suspicion for CLN2 disease is low. The majority responded that patient referral to a knowledgeable specialist can take longer than one year and identified delays in clinical suspicion of CLN2 as a critical barrier to diagnosis. On average, a 2–3 year delay exists between first onset of symptoms and diagnosis. 84% of experts performed either a TPP1 enzyme activity test and/or a molecular test to confirm diagnosis. The group described the gold standard for diagnosis as demonstration of decreased TPP1 enzyme activity and/or detection of two pathogenic mutations in the TPP1/CLN2 gene. Initial presenting symptoms were identified as epilepsy/seizures (86%), speech decline (64%), and delay/regression in development (50%). CLN2 symptoms were reported to develop between 18 months and 5 years of age most commonly, although 23% of experts noted that some patients present with symptoms later in life. Experts agreed that the most impactful strategies in managing patients with CLN2 disease were use of antiepileptic drugs (100%), gastric tubes (86%), and physical/occupational therapy (57%). Despite this management approach, 67% thought CLN2 patients' seizures were relatively treatment-resistant. Conclusion CLN2 disease is a severe, progressive neurodegenerative disorder: patients typically experience uncontrolled seizures, rapid deterioration of motor and cognitive functions, gradual loss of vision, and premature death. Disease awareness is low among non-expert clinicians, resulting in delays in diagnosis and referrals. These survey data suggest that worldwide education regarding CLN2 disease is necessary to support timely diagnosis so that future therapeutic options may be initiated early in the disease course.

Psychiatric consultations and the management of associated comorbid medical conditions in a regional referral hospital

<p><strong>Background</strong>. Psychiatrists are often called upon to evaluate patients with a medical condition and psychiatric symptoms, either as a complication thereof or initial presenting symptoms. There are often grey areas with regard to neuropsychiatric disorders in which psychiatrists and specialists from other clinical disciplines would need to co-manage or share ideas on the comprehensive treatment of a presenting patient. Objectives. This study was undertaken to provide a demographic and clinical profile of all patients consulted by the consultation-liaison psychiatry (CLP) service at the Helen Joseph Hospital (HJH) in Johannesburg, and to describe the clinical management of patients admitted with a diagnosis of a mental disorder associated with a comorbid medical condition, including delirium, dementia and a mood or psychotic disorder due to a general medical condition.</p><p><strong> Methods.</strong> A retrospective record review of all patients referred to the HJH CLP team over a 6-month period. Results. A total of 884 routine and emergency consultations were done for 662 patients (males n=305; females n=357) between the ages of 13 and 90 years who were referred from various other clinical departments. The most common documented reason for referral was a request for assessment (n=182; 27.5%), which consisted of mental state assessment, reconsultation and assessing capacity. A total of 63 patients (10.0% of cases consulted) were admitted to either the medical or psychiatric wards with a confirmed diagnosis of delirium, dementia and/or a mood or psychotic disorder due to a general medical condition (although admission wards were identified in 55 files only). The medical wards admitted the majority (n=37; 67.3%) mostly for delirium (n=28; 50.9%). HIV was identified as the most common systemic aetiological factor (n=23; 67.7%).</p><p><strong> Conclusion.</strong> In this study, a female patient between 31 and 45 years of age was slightly more likely to be referred to the HJH CLP service for assessment, and was more likely to be managed in the medical wards for delirium, which was most often associated with HIV/AIDS. The study highlighted the need for development of guidelines to facilitate adequate and effective use of this service for the local practice of CLP in a general specialist referral hospital like HJH, which would cover the following: clinical management; training needs; and administrative procedures.</p>

Targeted screening for the detection of Pompe disease in patients with unclassified limb-girdle muscular dystrophy or asymptomatic hyperCKemia using dried blood: A Spanish cohort

We aimed to screen for Pompe disease in patients with unclassified limb-girdle muscular dystrophy (LGMD) or asymptomatic hyperCKemia using dried blood spot (DBS) assays. Subsequently, we aimed to calculate the diagnostic delay between initial symptom presentation and the diagnosis. A prospective, multicenter, observational study was conducted in 348 patients: 146 with unclassified LGMD and 202 with asymptomatic or paucisymptomatic hyperCKemia. We quantified levels of acid alpha-glucosidase (GAA) from dried blood spots analyzed fluorometrically. The test was positive in 20 patients, and Pompe disease was confirmed by genetic testing in 16. Undiagnosed Pompe disease was detected in 7.5% of patients with LGMD and in 2.5% of patients with persistent, idiopathic elevation of serum creatine kinase. The c.-32-13 T > G mutation was found most commonly. The diagnostic delay was 15 years on average. In conclusion, DBS tests are useful and reliable screening tools for Pompe disease. We recommend the dried blood spot test to be included in the diagnostic work-up of patients with unclassified myopathies with proximal weakness and/or hyperCKemia of unknown cause and, when positive, to define the diagnosis, it will have to be confirmed by biochemical and/or molecular genetic analysis.

Gait Disturbance as the Initial Presenting Symptom in Young Children With Anti-NMDA Receptor (Anti-NMDAR) Encephalitis: A Case Series (I4-5C)

Gait Disturbance as the Initial Presenting Symptom in Young Children With Anti-NMDA Receptor (Anti-NMDAR) Encephalitis: A Case Series (I4-5C)

Gait Disturbance as the Initial Presenting Symptom in Young Children With Anti-NMDA Receptor (Anti-NMDAR) Encephalitis: A Case Series (P6.263)

Gait Disturbance as the Initial Presenting Symptom in Young Children With Anti-NMDA Receptor (Anti-NMDAR) Encephalitis: A Case Series (P6.263)

Clinical outcomes in patients with acute eosinophilic pneumonia not treated with corticosteroids.

The objective of this study was to evaluate the course of clinical stability in patients with acute eosinophilic pneumonia (AEP) who did not receive corticosteroid treatment. Secondary analysis included 19 consecutive patients with AEP who did not receive corticosteroid treatment from a cohort of 310 patients newly diagnosed with AEP between October 2007 and December 2013. All patients presented with dyspnea, fever, or cough with diffuse pulmonary infiltration. All but one patient (95 %) had elevated C-reactive protein (CRP), and 11 (58 %) patients had peripheral eosinophilia at the time of diagnosis. During the follow-up period, the dyspnea improved within a median of 4 (3-6) days and defervescence occurred within a median of 5 (4-7) days. Median time to clinical stability (defined as disappearance of all initial presenting symptoms) was 9 (7-12) days. In addition, the majority of pulmonary infiltrates on chest radiographs completely disappeared within 14 days after diagnosis. However, the peripheral eosinophil count and the frequency of peripheral eosinophilia increased up to 10 days and then decreased during the follow-up period. All patients experienced peripheral eosinophilia during hospitalisation. AEP-associated symptoms and radiographic abnormalities were resolved completely within 2 weeks after diagnosis even when corticosteroid treatment was not initiated. However, these findings might be limited to relatively mild cases of AEP.

Detection of human exposure to saxitoxin and neosaxitoxin in urine by online-solid phase extraction-liquid chromatography-tandem mass spectrometry.

Saxitoxin (STX) and neosaxitoxin (NEO) are potent neurotoxins that cause paralytic shellfish poisoning (PSP). PSP typically occurs through the ingestion of bivalve shellfish that have consumed toxin producing dinoflagellates. Due to initial presentation of symptoms being nonspecific, a clinical measurement is needed to confirm exposure to these toxins. Our group has developed an online solid phase extraction hydrophilic interaction liquid chromatography (HILIC) method for the analysis of STX and NEO in human urine with tandem mass spectrometry. A unique feature of this online method is the incorporation of a new synthetic (15)N4-STX labeled internal standard used for quantitation. Manual sample preparation time was reduced by approximately 70% for 98 urine samples as compared to a previously reported method. The lowest reportable limit for STX was improved from 5.0ng/mL to 1.01ng/mL and from 10.0ng/mL to 2.62ng/mL for NEO. Three analysts validated the method with 20 calibration curves total over 30 days with precision and accuracy within ±15% for all QCs. This new online method rapidly identifies STX and NEO exposure with improved sensitivity, which can facilitate the work of public health authorities to confirm the cases of PSP, complementing the many shellfish monitoring programs worldwide.

Concomitant anterior and posterior urethral valves in pediatrics: A single center experience over 12 years and long-term follow up after endoscopic treatment.

To report our 12-year experience with endoscopic management of patients with concomitant anterior and posterior urethral valves. We retrospectively reviewed the charts of patients referred to us for management of urethral valves from 2000 to 2012 to find cases with concomitant anterior and posterior valves. The diagnosis of valves was first suspected on voiding cystourethrography and confirmed by urethrocystoscopy. We collected available data on patients' age at diagnosis, clinical presentations, ultrasound and urodynamic findings, and surgical treatments. The final outcome at last follow up was also recorded. From 38 cases with anterior urethral valve, six (15.8%) presented concomitant anterior and posterior valves. The age at diagnosis in these patients ranged from antenatal diagnosis to 13 years. Initial presenting symptoms were recurrent urinary tract infection, incontinence, urosepsis and poor urinary stream. All valves were ablated by transurethral fulguration/resection using small-sized urethrocystoscopes. Among those with concomitant anterior and posterior valves, four patients had vesicoureteral reflux at presentation that resolved in two patients after valve ablation. One patient progressed to renal failure and required dialysis. Bladder hypercontractility and detrusor overactivity were the main urodynamic patterns in these patients. Concomitant anterior and posterior valves seem to be more prevalent than previously assumed, and might be missed on initial assessment. Oblique view voiding cystourethrography with full-length delineation of the urethra is of paramount diagnostic importance when obstruction is suspected. A meticulous urethrocystoscopy should follow for confirming the diagnosis and endoscopic ablation/resection of the valves.

Giant Petrous Bone Osteoma Associated With Ipsilateral Clivus Meningioma in an Adult

Background and Importance: An association with multiple primary intracranial tumors of different histologic types not relating to radiotherapy or phacomatosis is a rare event. To the best of the authors’ knowledge, a giant osteoma originating from the inner surface of the petrous bone associated with an ipsilateral clivus meningioma has never been reported. Clinical Presentation: A 43-year-old patient with trigeminal neuralgia was found to have a posterior fossa bony lesion measuring 4 cm, with a solid enhancing lesion in juxtaposition and a separate bony lesion on the occipital bone on magnetic resonance and computed tomography imaging. A satisfactory resection of the coexisting tumors in the petroclival region was performed through a subtemporal transtentorial approach. The tumors were finally confirmed as an osteoma and a meningioma by pathologic evaluation. The patient demonstrated an improvement in her trigeminal neuralgia over a 3-month postoperative period of time. Conclusions: This case presents new clinical and radiographic findings associated with a rare lesion. Dependent tumors that one tumor may act as an irritating agent for the local proliferation of the other most likely accounted for this pattern of tumoral linkage. A satisfactory surgical result was achieved through a relatively minimal approach and different strategies. Trigeminal neuralgia, the initial presenting symptom, improved during the follow-up period.

Multicentric Castleman's Disease in a Child Revealed by Chronic Diarrhea.

Multicentric Castleman's disease is a rare benign and unexplained lymphoproliferative disorder that is extremely uncommon in children. It presents with fever, systemic symptoms, generalized lymphadenopathy, and laboratory markers of inflammation. Its treatment is not standardized and its prognosis is poor. We report a novel case of multicentric Castleman's disease in a 13-year-old girl who had presented with chronic diarrhea as the only initial presenting symptom. The diagnosis of celiac or inflammatory bowel diseases was suspected, but two and a half years later, the diagnosis of multicentric Castleman's disease was brought following the appearance of abdominal mass whose biopsy revealed Castleman's disease in the plasma cell form. The outcome was favorable after treatment by corticosteroid, chemotherapy, and surgery. The occurrence of diarrhea as the initial symptom of multicentric Castleman's disease without lymph node involvement is very rare. This case report underlines the diagnostic difficulties and the long interval between onset and diagnosis when diarrhea occurs first.

Primary spinal epidural cavernous hemangioma: clinical features and surgical outcome in 14 cases.

The aim of this study was to investigate the clinical characteristics, imaging features, differential diagnosis, treatment options, and prognosis for primary spinal epidural cavernous hemangiomas. Fourteen patients with pathologically diagnosed non-vertebral origin cavernous hemangiomas who had undergone surgery at Beijing Tiantan Hospital between 2003 and 2012 were identified in the hospital's database. The patients' clinical data, imaging characteristics, surgical treatment, and postoperative follow-up were analyzed retrospectively. There were 9 males and 5 females with an average age of 51.64 years. The primary epidural cavernous hemangiomas were located in the cervical spine (2 cases), cervicothoracic junction (2 cases), thoracic spine (8 cases), thoracolumbar junction (1 case), and lumbar spine (1 case). Hemorrhage was confirmed in 4 cases during surgery. Preoperatively 5 lesions were misdiagnosed as schwannoma, 1 was misdiagnosed as a meningioma, and 1 was misdiagnosed as an arachnoid cyst. Preoperative hemorrhages were identified in 2 cases. Three patients had recurrent cavernous hemangiomas. The initial presenting symptoms were local pain in 5 cases, radiculopathy in 6 cases, and myelopathy in 3 cases. Upon admission, 1 patient had radicular symptoms and 13 had myelopathic symptoms. The average symptom duration was 18 months. All patients underwent surgery; complete resection was achieved in 8 cases, subtotal resection in 4 cases, and partial resection in 2 cases. Postoperative follow-up was completed in 10 cases (average follow-up 34 months); 1 patient died, 5 patients showed clinical improvement, and 4 patients remained neurologically unchanged. Total surgical removal of spine epidural cavernous hemangiomas with a chronic course is the optimum treatment and carries a good prognosis. Secondary surgery for recurrent epidural cavernous hemangioma is technically more challenging. In patients with profound myelopathy from acute hemorrhage, even prompt surgical decompression can rarely reverse all symptoms.