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Objective:To investigate the clinical and imaging differences between serum aquaporin 4 (AQP4) antibody positive and negative patients with neuromyelitis optica spectrum disorder (NMOSD).Methods:The clinical data and radiologic findings of 89 NMOSD patients diagnosed at Beijing Tiantan Hospital, Capital Medical University from January 2018 to June 2022 were retrospectively analyzed. There were 17 male cases and 72 female cases, aged 18-74 years. According to the results of serum AQP4 antibody test, the patients were divided into AQP4 antibody positive group and AQP4 antibody negative group, and the differences in clinical data, lesion distribution, lesion characteristics, and brain area volume between the 2 groups were compared using independent sample t-test and χ 2 test, and the correlation between brain area volume and expanded disability status scale (EDSS) scores was further investigated using Spearman correlation analysis. Results:There were 68 cases in the AQP4 antibody positive group and 21 cases in the AQP4 antibody negative group. Patients in both groups were predominantly female, but the percentage of females in the AQP4 antibody-positive group (86.8%, 59/68) was higher than that in the AQP4 antibody-negative group (61.9%, 13/21), with a statistically significant difference (χ 2=4.91, P=0.027). The incidence of optic neuritis in AQP4 antibody negative group (66.7%, 14/21) was higher than that in antibody positive group (41.2%, 28/68), with a statistically significant difference (χ 2=4.18, P=0.041). In the distribution of intracranial lesions on MRI, the probability of lesions involving the brain stem in AQP4 antibody negative group (47.6%, 10/21) was higher than that in AQP4 antibody positive group (23.5%, 16/68), the difference had statistically significance (χ 2=4.50, P=0.034). The volumes of whole brain white matter, right amygdala, right accumbens-area and right ventral diencephalon in AQP4 antibody positive group were lower than those in AQP4 antibody negative group ( P<0.05), and the volumes of the right accumbens-area were negatively correlated with the EDSS scores in AQP4 antibody positive group ( r=-0.628, P=0.009). Conclusion:There are differences in clinical and imaging manifestations between AQP4 antibody positive and AQP4 antibody negative patients, which provides more basis for clinical in-depth understanding of NMOSD.
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Purpose: To elucidate the clinico?epidemiologic characteristics of optic neuritis based on the status of serum aquaporin?4 antibody (AQP4?Ab) in patients with optic neuritis (ON). Methods: Medical records of 106 patients with ON and a follow?up of 3 years were reviewed. For each patient, the following data were extracted: medical history, findings of the ocular examination, brain, orbital or spinal MRI, and serological tests for AQP4. The ON was classified as typical or atypical based on disc examination and improvement in vision after intravenous methylprednisolone (IVMP). The clinical findings (typical or atypical), disease course, and outcomes were analyzed according to the serostatus of the ON. Results: 10 patients ((9.4%) were seropositive for AQP4?Ab; all had atypical ON. 96 patients (91%) were seronegative for AQP4?Ab: 36 atypical ON and 60 typical ON. Profound visual impairment at presentation was seen in all patients. However, at the end of the study period, seropositive and seronegative atypical ON had poor visual outcomes as compared to seronegative typical ON (P = 0.002). Five seropositive and four seronegative patients with atypical ON developed transverse myelitis. Bilateral disease with relapse was more in seropositive patients (80%); however, seronegative with atypical ON also had bilateral presentation and relapse in 42% and 41%, respectively. Conclusion: AQP4?Ab seropositive patients mostly present with atypical features such as bilateral recurrent ON, poor visual outcome, and increased incidence of transverse myelitis. However, atypical clinical features can also be seen in seronegative ON with a poor visual outcome and a recalcitrant course.
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Objective: Neuromyelitis optica spectrum disorder (NMOSD) is an inflammatory disease of the central nervous system characterized by optic neuritis and myelitis. NMOSD is more prevalent in the elderly than multiple sclerosis. In particular, optic neuritis of NMOSD is common in the elderly.Methods and Results: We report an 82-year-old female patient with no medical history who presented with optic neuritis as the first attack of NMOSD. On day X−15, she developed horizontal hemianopia and was referred to our department. On admission, her visual acuity was hand motion. Cerebrospinal fluid suggested pleocytosis. Magnetic resonance imaging revealed a hyper-intense lesion on the optic chiasm and optic tract. Steroid pulse therapy was performed, but from the night, delirium with visual hallucinations developed. She refused drug administration, removed the intravenous administration route, and was unable to continue in-hospital treatment.Conclusion: NMOSD often develops in the elderly with optic neuritis, and in addition to steroid therapy, plasma exchange or immunoglobulin therapy is expected to improve the prognosis. However, in our case, treatment was discontinued due to marked delirium accompanied by visual hallucinations. Visual hallucinations are more likely to occur in elderly patients with visual impairment, and clinicians should be careful and manage hallucination-associated delirium.
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Objective@#To compare the changes of spontaneous brain activity in myelin oligodendrocyte glycoprotein antibody (MOG-Ab) positive and Aquaporin 4 antibody (AQP4-Ab) positive neuromyelitis optica spectrum disorder (NMOSD) by using resting-state functional magnetic resonance imaging (fMRI).@*Methods@#A case control study was designed.A total of 11 NMOSD patients with positive MOG-Ab and 21 NMOSD patients with positive AQP4-Ab were enrolled from October 2006 to May 2017 in PLA General Hospital.Thirty-four healthy controls closely matched in age, sex and education were recruited and underwent resting-state fMRI scans.The amplitude of low-frequency fluctuation (ALFF) was extracted to investigate the spontaneous brain activity.This study was approved by Ethics Committee of PLA General Hospital (S2019-111-01). All subjects enrolled signed informed consent.@*Results@#Two patients in the MOG-Ab positive group had seizure history, and no seizure history was observed in AQP4-Ab positive group and healthy control group.Compared with healthy control group, all patients in MOG-Ab positive group and AQP4-Ab positive group had significantly increased ALFF values of prefrontal gyrus.The ALFF values of bilateral anterior central gyrus and bilateral posterior central gyrus in AQP4-Ab positive group were 1.89±0.56 and 2.10±0.69, respectively, which were lower than 3.32±1.15 and 3.61±1.23 in MOG-Ab positive group, the differences were statistically significant (both at P<0.001, AlphaSim correction).@*Conclusions@#Resting-state fMRI could provide new evidence of possibly multi-focal disease mechanisms.Hyperactivity in prefrontal cortex, motor cortex and somatosensory cortex might reflect differences in pathological processes between MOG-Ab positive and AQP4-Ab positive NMOSD patients.
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@#Background & Objective: To evaluate the prevalence of aquaporin-4 antibody (AQP-4 Ab) seropositive patients in neuromyelitis optica (NMO) and neuromyelitis optica spectrum disorder (NMOSD) and to compare the clinical, investigation features and treatment outcome between the AQP-4 Ab seronegative and seropositive groups. Methods: All NMO and NMOSD patients in Maharat Nakhon Ratchasima hospital during January 2012 to December 2016 were recruited. Demographic, clinical, laboratory and imaging data were collected from the medical records. All data were analysed and compared between seropositive and seronegative groups as appropriates. Results: There were 12 (29%) and 30 (71%) NMO and NMOSD patients, respectively. There were 30 (71%) patients who hadAQP-4 Ab seropositive status. Thirty-three (78.6%) patients were female. In seropositive group, there were significantly higher proportion of female patients (87% versus 58%, p=0.04), immunosuppressant treatment (33% versus 0%, p=0.04), patients who had serum albumin less than 4 g/dL (46% versus 0%, p=0.02), cerebrospinal fluid (CSF) pleocytosis (71% versus 17%, p=0.01) and patients with extensive spinal cord involvement (67% versus 25%, p<0.05) than seronegative group. CSF-serum glucose ratio was significantly lower in seropositive group than seronegative group (0.5 + 0.03 versus 0.7 + 0.04, p=0.01). Conclusion: The prevalence of AQP-4 Ab seropositive patients in NMO and NMOSD was 71%. There were significantly more female patients, immunosuppressant treatment, patients with serum albumin less than 4 g/dL, CSF pleocytosis, low CSF-serum glucose ratio and extensive transverse myelitis than seronegative group.
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Objective To investigate the clinical and imaging features of the aquaporin-4 (AQP4)antibody-negative binocular optic neuritis and to analyze the predictive factors of visual function outcome.Methods Fifty-eight patients with AQP4-negative binocular optic neuritis were reviewed and followed up from January 2014 to December 2015.Patients at baseline and at the end of follow-up were evaluated for visual function and neurological examination.All patients underwent optic nerve and brain MRI, cerebrospinal fluid and routine laboratory tests.Results AQP4 antibody-negative binocular optic neuritis accounted for 9.4%(58/615)of the total optic neuritis in the same period.At baseline, 99 eyes (85.3%,99/116)had best corrected visual acuity<0.1.At the end of follow-up, 31 eyes(26.7%,31/116) had best corrected visual acuity < 0.1. There were 43 cases (74.1%, 43/58) with multi-segment involvement of optic nerve at the baseline.Baseline visual acuity(P=0.005), early treatment response (P=0.011), and segment numbers of optic nerve involvement(P=0.025)were independently associated with end-point outcome of visual function.Forty-nine patients(84.5%,49/58)showed monophasic course in (3.1 ± 0.9) years follow-up period, 7 cases (12.1%, 7/58) had recurrence, and 2 cases (3.4%, 2/58) converted to neuromyelitis optic spectrum disorder (NMOSD). Conclusions AQP4 antibody-negative binocular optic neuritis is common and the recovery of visual function is not satisfied. Baseline visual function and the length of optic lesion in MRI is related to the end-point prognosis. Most patients performs the single phase course during the follow-up period.
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<p><b>Background:</b> Chronic subdural hematoma (CSDH) is often found in the elderly owing to slight head trauma and is associated with several neurological disorders. Neurological deficits are cured by a simple surgical removal of the hematoma; however, these deficits persist if there is insufficient hematoma removal. It is rare for patients to continue having neurological disorders once the hematoma is removed.</p><p><b>Case report:</b> A 61-year-old woman presented with gait disturbance. She was diagnosed with a subdural hematoma through head computed tomography. After hematoma irrigation, her gait disturbance exacerbated, and she developed urinary tract dysfunction. Ubiquitous neurodegeneration in the midbrain and spinal cord was suspected owing to a hyperintense signal on fluid-attenuated inversion recovery of magnetic resonance imaging. The anti-aquaporin 4 antibody was detected in the patient’s serum, and she was diagnosed with neuromyelitis optica (NMO).</p><p><b>Conclusions:</b> Progressive NMO caused gait dysfunction and triggered head trauma, followed by CSDH. Although NMO rarely causes CSDH, it should be considered in uncommon cases of CSDH.</p>
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According to the sera aquaporin-4 antibody (AQP4), neuromyelitis optica (NMO) can be classified into two types as AQP4 antibody positive (AQP4+) and negative (AQP4-). However, the NMO patients with AQP4- are prone to delayed treatment, and may have a different pathogenesis compared to that in patients with AQP4+. Scientific researches and the clinical trials on NMO with AQP4- will deepen the understanding of NMO pathogenesis and help to make an early accurate diagnosis and rational therapy for NMO with AQP4-. This review aims to summarize the progress in clinic diagnosis for NMO patients with AQP4-.
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Objective:To investigate the relationship between anti-Helicobacter pylori antibody(Hp-IgG)and anti-aquaporin 4 antibody which are in neuromyelitis optica(NMO)and multiple sclerosis(MS).Methods: Serum specimens were collected from the 33 patients with MS,7 patients with NMO,and 35 health examination cases.Hp-IgG were detected by enzyme-linked immunosorbent assasy and anti-aquaporin 4 antibody were detected by cell based assay respectively.The positive rate of Hp-IgG and anti-aquaporin 4 antibody were analyzed,and the difference of Hp-IgG positive rate was compared between patients with Hp-IgG positive and negative.Results: Serum Hp-IgG positive rate of MS,NMO and normal control groups were 69.70％,85.71％ and 42.86％ respectively with a significant statistically difference of Hp-IgG(P0.05).Serum anti AQP4 antibody positive rate of MS,NMO and normal control groups were 4.2％,85.71％ and 0％ respectively with a significant statistically difference of anti AQP4 antibody(P0.05).Conclusion: HP infection is a risk factor for the occurrence of MS and NMO,but not associated with MS and NMO patients with anti AQP4 antibodies.
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Objective To determine the correlation between aquaporin-4 antibody (AQP4-Ab) and optic neuropathy in patients with neuromyelitis optica spectrum disorder (NMOSD).Methods The clinical and biochemical data of 53 patients with NMOSD diagnosis based on AQP4-Ab level in Changhai Hospital between January 2010 and October 2015 were retrospectively analyzed.According to optic neuropathy occurrence,the NMOSD patients were divided into optic neuropathy and non-optic neuropathy groups.Clinical and biochemical characteristics were compared between the two groups.According to the serum AQP4-Ab levels,the NMOSD patients were divided into AQP4-Ab seropositive and seronegative groups.The incidence of optic neuropathy was compared between the two groups.The correlation between optic neuropathy and AQP4-Ab levels was analyzed.Results Between the optic neuropathy and non-optic neuropathy groups,no significant differences in sex,age at onset,disease course,serum alanine aminotransferase levels,protein levels in cerebral spinal fluid,IgG index,and oligoclonal band were observed (P ＞ 0.05).However,statistically significant differences were found in frequency,superficial sensory impairment,serum creatinine level,and serum AQP4-Ab level (P ＜ 0.05).Between the AQP4-Ab sempositive and semnegative groups,a statistically significant difference in the incidence of optic neuropathy was observed (F =4.93,P ＜ 0.05).The incidence of optic neuropathy positively correlated with AQP4-Ab levels (r =0.297,P ＜ 0.05).Conclusion NMOSD patients with AQP4-Ab seropositivity could be prone to optic neuropathy,and the correlation may be beneficial to early diagnosis,therapy,and monitoring of NMOSD.
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Objective To explore the relationship between the damage of neuromyelitis optica (NMO) astrocytes (AS) and the onset of NMO, and investigate the relevance of AS damage with the severity of the patients with functional defect. Methods The levels of aquaprin4-antibody (AQP4 -Ab), glial fibrillary acidic protein (GFAP), apolipoprotein(ApoE),interleukins-6(IL-6), interleukins-10(IL-10), tumor necrosis factor-α(TNF-α) in cerebrospinal fluid (CSF ) and serum of 30 acute NMO patients were tested by means of ELISA. The results were later compared with control group. And analysis of the relevance of the various index of the levels in CSF with the CSF AQP4-Ab level, the acute phase expanded disability status scale(EDSS) score of the NMO group were made. Results (1)The NMO group in CSF and serum AQP4-Ab, GFAP, IL-6 levels were higher than the control group (P < 0.05), and ApoE, IL-10 levels were lower than the control group (P < 0.05). (2)The CSF GFAP, ApoE, IL-6 in NMO group is higher than the serum (P < 0.05), and CSF AQP4-Ab, IL-10 levels were lower than the serum ( P < 0 . 05 ) . ( 3 ) The CSF GFAP , IL-6 levels and the CSF AQP4-Ab level were positively correlated (r=0.749, r=0.526, P<0.05), and the CSF ApoE, IL-10 levels were negatively correlated with CSF AQP4-Ab level(r = -0.571, r = -0.676, P < 0.05). (4)The CSF AQP4-Ab, GFAP, IL-6 levels and the acute phase EDSS score were positively correlated (P < 0.05), the CSF ApoE, IL-10 levels were negatively correlated with the acute phase EDSS score (P < 0.05). Conclusion The AS damage exists in the NMO and the damage severity may correlate with patient function defect. AQP4-Ab, GFAP, IL-6 may play important roles in the onset of NMO and the disease aggravating. The decrease of the ApoE and IL-10 may exacerbate NMO damage.
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AIM:To investigate the serum antibody of aquaporin 4 ( AQP4 - Ab ) in positive expression rate and the significance in patients with neuritis. ●METHODS: A total of 98 cases ( 128 eyes ) of patients with optic neuritis were studied to detect the patient′s serum AQP4-Ab positive rate of antinuclear antibodies ( ANAs) from Jan. 2012 to Dec. 2015 in ophthalmology center of our hospital. According to the expression of AQP4 - Ab group, the best corrected visual acuity between the two groups, peripapillary nerve fiber layer thickness (pRNFL), the volume of the macula, macular RNFL ( mRNFL ) , macular core layer volume ( mlNL ) measurement were compared. ●RESULTS:Ninety-eight patients (128 eyes) with optic neuritis cases diagnosed through examination revealed AQP4-Ab positive in 22 patients ( 22%) , negative in 76 patients ( 78%) , ANAs positive in 21 patients ( 21%) , negative 77 patients ( 79%) . Optic neuritis patients with serum AQP4 - Ab positive rate and ANAs positive significant correlation ( r = 0. 707, P 0. 05). After inspection found pRNFL, macular volume measured value AQP4 - Ab positive patients were significantly less than the negative patients, the differences were statistically significant (P0. 05). ●CONCLUSION:AQP4-Ab and ANAs expression in optic neuritis patients is a significant correlation. AQP4-Ab positive patients with optic neuritis pRNFL thinning of macular volume are decreased compared with negative patients.
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O bloqueio do TNF tem tido sucesso no tratamento de algumas doenças reumáticas, como a espondiloartrite. Relatam-se muitas complicações infecciosas com a terapia anti-TNF, principalmente infecções bacterianas, micobacterianas, virais e fúngicas. A Entamoeba histolytica é um protozoário extracelular que causa principalmente colite e abscesso hepático, sendo que a perfuração intestinal é uma complicação rara, com alta mortalidade. O TNF é considerado o principal mediador da imunidade celular contra a amebíase. Inicialmente, é quimiotático para a E. histolytica, potencializando sua adesão ao enterócito por meio da lectina galactose-inibível, e depois ativando os macrófagos para matarem a ameba pela liberação de NO; assim, o bloqueio do TNF poderia ser prejudicial, aumentando a virulência amebiana. Descreve-se o caso de uma mulher de 46 anos com espondiloartrite que apresentou uma perfuração do colo por colite amebiana invasiva durante uso de anti-TNF.
Optic neuritis (ON) was rarely reported in juvenile idiopathic arthritis (JIA) patients, particularly in those under anti-tumor necrosis factor alpha blockage. However, to our knowledge, the prevalence of ON in JIA population has not been studied. Therefore, 5793 patients were followed up at our University Hospital and 630 (11%) had JIA. One patient (0.15%) had ON and was reported herein. A 6-year-old male was diagnosed with extended oligoarticular JIA, and received naproxen and methotrexate subsequently replaced by leflunomide. At 11 years old, he was diagnosed with aseptic meningitis, followed by a partial motor seizure with secondary generalization. Brain magnetic resonance imaging (MRI) and electroencephalogram showed diffuse disorganization of the brain electric activity and leflunomide was suspended. Seven days later, the patient presented acute ocular pain, loss of acuity for color, blurred vision, photophobia, redness and short progressive visual loss in the right eye. A fundoscopic exam detected unilateral papilledema without retinal exudates. Orbital MRI suggested right ON. The anti-aquaporin 4 (anti-AQP4) antibody was negative. Pulse therapy with methylprednisolone was administered for five days, and subsequently with prednisone, he had clinical and laboratory improvement. In conclusion, a low prevalence of ON was observed in our JIA population. The absence of anti-AQP4 antibody and the normal brain MRI do not exclude the possibility of demyelinating disease associated with chronic arthritis. Therefore, rigorous follow up is required.
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Humans , Male , Child , Arthritis, Juvenile/complications , Optic Neuritis/etiologyABSTRACT
BACKGROUND AND PURPOSE: Although plasmapheresis is becoming standard practice as a rescue therapy for neuromyelitis optica (NMO), evidence for the therapeutic efficacy of plasmapheresis is limited, and the effect of plasmapheresis on anti-aquaporin-4 (AQP4) levels in patients with NMO has not been reported. Here, our objective was to evaluate the clinical efficacy of therapeutic plasmapheresis and its effect on anti-AQP4 antibody levels in patients with NMO spectrum disorder (NMOSD). METHODS: We retrospectively reviewed the medical records of 15 patients with NMOSD who had 18 acute attacks and received plasmapheresis because they did not respond to high-dose intravenous methylprednisolone (IVMP) therapy. Anti-AQP4 antibodies were measured before and after plasmapheresis. The primary outcomes were functional improvements immediately and 6 months after plasmapheresis, and the secondary outcome was the change in anti-AQP4 antibody serum levels following plasmapheresis. RESULTS: Plasmapheresis following IVMP therapy led to significant improvement in 50% of the 18 attacks in 15 patients immediately after the procedure was completed, and in 78% (14 attacks) after 6 months. Plasmapheresis was generally well tolerated in all patients. Anti-AQP4 antibody serum levels declined significantly following plasmapheresis, to a mean of 15% of the preplasmapheresis levels. Lower scores on the visual outcome scale recorded before an attack were associated with significant immediate improvement upon the completion of plasmapheresis (p=0.03). CONCLUSIONS: Plasmapheresis following IVMP therapy effectively removed anti-AQP4 antibodies and was accompanied by a substantial improvement in the neurological disability of patients with NMOSD. Lower levels of pre-existing neurological damage may be associated with an improved acute response to plasmapheresis.
Subject(s)
Humans , Antibodies , Medical Records , Methylprednisolone , Neuromyelitis Optica , Plasmapheresis , Retrospective StudiesABSTRACT
La presencia de anticuerpos IgG en suero, con blanco en los canales de acuaporina-4, es específica de la neuromielitis óptica (NMO). El 60% de los pacientes con NMO presentan lesiones cerebrales en la resonancia magnética (RM); en un 8% (mayoría niños) estas lesiones se consideraron "atípicas". Presentamos dos pacientes con NMO y lesiones en el SNC de alta expresión de acuaporina-4. Caso 1: varón de 50 años, que comenzó con pérdida de visión en ojo derecho (OD). Recibió tratamiento empírico con metilprednisolona 1 g/d x 3 días. Al mes presentó dolor generalizado y hemiparesia derecha; nuevamente recibió metilprednisolona 1 g/d x 5 días e IgG IV 400 mg/kg/d × 5 días. Recuperó la deambulación persistiendo el dolor y fenómenos paroxísticos en los 4 miembros. Potenciales evocados visuales: P100, ojo izquierdo (OI) 123 mseg. OD sin respuesta. La RM de cerebro (FLAIR) mostró hiperintensidad en nervio óptico derecho, hipotálamo y comisura blanca anterior. RM cervical: lesión medular extensa (5 cuerpos vertebrales). Caso 2: mujer de 53 años, con disminución de la agudeza visual en ambos ojos y parestesias en miembros inferiores que remitieron espontáneamente. Evolucionó al mes con cuadriparesia e incontinencia esfinteriana. Recibió metilprednisolona 1 g/d x 5 días, sin mejoría. Potenciales evocados visuales: P100 OI 124 mseg. OD 128 mseg. RM cerebro: (FLAIR) hiperintensidad hipotalámica y periacueductal. RM cervical: lesión medular extensa (7 cuerpos vertebrales). Anticuerpos anti-acuaporina-4 positivos en ambos pacientes (inmunofluorescencia indirecta). Las lesiones consideradas "atípicas", como aquí, en sitios con alta densidad de proteínas canales de agua AQP4 deberán considerarse para el diagnóstico diferencial.
Disease-specific aquaporin-4 antibodies (NMO-IgG) are the main effector of lesions in neuromyelitis optica (NMO) patients. Brain MRI lesions are detected in 60% of them, with 8% (almost infants) at sites of high aquaporin-4 expression. Patient 1: A fifty-year-old male with loss of vision in the right eye. Empiric treatment with metilprednisolone 1g/d for 3 days was indicated. After 30 days he complained of generalized pain, and a right hemiparesis was evident. The patient received bolus of metilprednisolone 1g/d for 5 days plus IgG 400 mg/kg/d IV for 5 days. He recovered ambulation but persisted with pain and paroxysmal phenomena (Lhermitte). Visual Evoked Potentials (VEP): P100 left eye 123 ms, right eye without response. Brain MRI (FLAIR) showed hyperintensity in the right optic nerve, hypothalamus and anterior white commissure. Cervical MRI showed extensive spinal cord lesion to an extension of 5 vertebral bodies. Patient 2: A fifty-three-year-old female who referred decreased visual acuity in both eyes and paresthesia in lower limbs which subsided spontaneously. One month later the patient evolved with cuadriparesis and sphincter incontinence. No improvement was observed with bolus of metilprednisolone 1g/d for 5 day. VEP: P100 left eye 124 ms, right eye 128 ms. Brain MRI (FLAIR) disclosed hypothalamic and periaqueductal hyperintensity. Cervical MRI showed extensive spinal cord lesion to an extension of 7 vertebral bodies. NMO-IgG antibodies were positive in both patients (indirect immunofluorescence assay). NMO brain lesions at sites of high aquaporin-4 expression, once considered "atypical" for their topography and infrequency in adults, should be borne in mind when considering differential diagnosis.