A child with Gradenigo syndrome presenting with meningism: a case report.

BACKGROUND: The symptoms of meningitis which include fever, headache, photophobia and irritability along with abducens nerve palsy pose a diagnostic dilemma requiring urgent attention. Here we report how such a dilemma was methodically and sequentially resolved using anatomical knowledge supported by neuroimaging and the eventual diagnosis of Gradenigo syndrome was made. CASE PRESENTATION: A 6-year-old previously healthy boy from Sri Lanka presented with high grade fever, headache, photophobia and left eye pain for 10 days and diplopia for 2 days duration. Neurological examination was unremarkable except for left sided abducens nerve palsy. He had high inflammatory markers and white blood cell count. A tentative differential diagnosis of acute bacterial meningitis complicated by cerebral oedema, acute hydrocephalus or cerebral abscess was made. However, non-contrast CT brain, cerebrospinal fluid analysis and electroencephalogram were normal leading to a diagnostic dilemma. MRI brain with contrast performed 3 days later due to limited resources revealed left mastoiditis extending to petrous temporal bone confirming Gradenigo syndrome. CONCLUSION: This case report highlights the importance of a thorough physical examination in children presenting with unrelated neurological symptoms and signs. Unilateral abducens nerve palsy raises the suspicion of increased intracranial pressure and neuroimaging is vital in diagnostic uncertainties. Gradenigo syndrome emphasises the importance of incorporating anatomical knowledge into clinical practice.

Neurological Manifestations of Mycoplasma pneumoniae Infection in Hospitalized Children and Their Long-Term Follow-Up.

Objective In this retrospective study, we aimed to assess frequency, types, and long-term outcome of neurological disease during acute Mycoplasma pneumoniae (M. pneumoniae) infection in pediatric patients. Materials and Methods Medical records of patients hospitalized with acute M. pneumoniae infection were reviewed. Possible risk factors were analyzed by uni- and multivariate regression. Patients with neurological symptoms were followed up by expanded disability status score (EDSS) and the cognitive problems in children and adolescents (KOPKJ) scale. Results Out of 89 patients, 22 suffered from neurological symptoms and signs. Neurological disorders were diagnosed in 11 patients: (meningo-) encephalitis (n = 6), aseptic meningitis (n = 3), transverse myelitis (n = 1), and vestibular neuritis (n = 1), 11 patients had nonspecific neurological symptoms and signs. Multivariate logistic regression identified lower respiratory tract symptoms as a negative predictor (odds ratio [OR] = 0.1, p < 0.001), a preexisting immune deficit was associated with a trend for a decreased risk (OR = 0.12, p = 0.058). Long-term follow-up after a median of 5.1 years (range, 0.6-13 years) showed ongoing neurological deficits in the EDSS in 8/18, and in the KOPKJ in 7/17. Conclusion Neurological symptoms occurred in 25% of hospitalized pediatric patients with M. pneumoniae infection. Outcome was often favorable, but significant sequels were reported by 45%.

URGENT DIAGNOSTICS OF CEREBRAL VENOUS TROMBOSIS.

Investigations were carried out in 24 patients in order to evaluate information value of the data of clinical, laboratory, neuroradiological methods of research and develop the diagnostic algorithm in case of cerebral venous thrombosis (CVT). The main group consisted of 11 patients (7 male, 4 female, average age 49,1±4,3) with CVT. The comparison group included 13 patients (6 male, 7 female; average age 68,1±9,5) with ischemic stroke (IS) of moderate severity. There were revealed changes in blood and cerebrospinal fluid (CSF) as form of leukocytosis of blood and moderately increased cell count with elevated protein in CSF and blood in case of CTV. The authors noted an elevated protein in CSF and blood and leukocytosis with predominant lymphopenia in blood and neurophilic predominance in CSF within the reference range of CSF in patient with ischemic stroke. The epileptic attacks, meningeal syndromes, headaches were more often among clinical syndromes at CTV than in case of ischemic stroke. The algorithm of neuroimaging research methods and modes of MRI were determined and allowed an effective diagnostics of damages of venous sinuses, superficial and deep cerebral veins in case of urgent hospitalization of patients. It was possible to suggest the venous pathology in 7 (63,6%) cases due to SKT (without contrast) and in case of application of MR venography (2D TOFmode), there were revealed 100% of cases.

Aneurysmal vasculopathy in human-acquired immunodeficiency virus-infected adults: Imaging case series and review of the literature.

BACKGROUND: Intracranial vasculopathy in adult patients with human-acquired immunodeficiency virus (HIV)/acquired immunodeficiency syndrome (AIDS) is a rare but increasingly recognized disease entity. OBJECTIVE: We aimed to contribute to and summarize the adult literature describing patients with HIV/AIDS who have intracranial vasculopathy. METHODS: A retrospective review of adult patients with HIV/AIDS undergoing diagnostic cerebral angiography at our institution from 2007-2013 was performed. A literature review of relevant existing studies was performed. RESULTS: Five adult patients with HIV-related aneurysmal and occlusive vasculopathy were diagnosed and/or treated at our institution. A comprehensive review of the literature yielded data from 17 series describing 28 adult patients with HIV/AIDS and intracranial vasculopathy. Our review suggests that low CD4 count, motor weakness, and meningismus may be associated with the sequelae of intracranial vasculopathy/vasculitis in patients with HIV/AIDS. CONCLUSION: Patients with HIV/AIDS who have aneurysmal and stenotic vascular disease may benefit from earlier surveillance with the onset of neurological symptoms. The roles of medical, open surgical, and endovascular therapy in this unique entity will be further defined as the pathological basis of the disease is better understood.

Safety and tolerability of gabapentin for aneurysmal subarachnoid hemorrhage (sah) headache and meningismus.

BACKGROUND: Headache after aneurysmal subarachnoid hemorrhage (SAH) is very common and is often described as the "worst headache imaginable." SAH-associated headache can persist for days to weeks and is traditionally treated with narcotics. However, narcotics can have significant adverse effects. We hypothesize that gabapentin (GBP), a non-narcotic neuropathic pain medication, would be safe and tolerable and would reduce narcotic requirements after SAH. METHODS: We retrospectively reviewed the clinical, radiographic, and laboratory data of SAH patients at the neuroscience intensive care unit at Mayo Clinic in Jacksonville, Florida, from January 2011 through February 2013. Headache intensity was quantified by a visual analog scale score. Total opioid use per day was tabulated using an intravenous morphine equivalents scale. Cerebrospinal fluid was also reviewed when available. RESULTS: There were 53 SAH patients who were treated with GBP along with other analgesics for headache. Among these SAH patients, 34 (64 %) were women, with a mean age of 54 years (SD 12.3). Severe headache was observed in all SAH patients. GBP dosing was rapidly escalated within days of SAH up to a median of 1,200 mg/day, with a range of 300 mg three times a day to 900 mg three times a day. Approximately 6 % of patients treated with GBP had nausea (95 % CI 1-16 %), and only one patient (1.8 %) had to discontinue GBP. CONCLUSIONS: GBP appears to be relatively safe and tolerable in SAH patients with headache and may be a useful narcotic-sparing agent to prevent narcotics-associated complications, such as gastrointestinal immobility, ileus, and constipation.

Outbreak of serotype W135 Neisseria meningitidis in central river region of the Gambia between February and June 2012: a hospital-based review of paediatric cases.

BACKGROUND: Meningitis still accounts for many deaths in children especially during epidemics in countries within the African meningitis belt. Between February and May 2012, the Gambia witnessed an outbreak of meningitis in two of its six regions. This study presents a clinical perspective of this outbreak in central river region of the Gambia. It evaluated the outbreak pattern, clinical features, and mortality among suspected cases that presented to the hospital during the outbreak. METHODOLOGY: This is a prospective observational study of suspected cases of meningitis that presented to the pediatric ward of the Bansang Hospital during the outbreak period. Confirmed cases of meningitis were consecutively enrolled, and those with negative blood cultures presenting during the same period were employed as controls. RESULT: Two hundred and four suspected cases of meningitis presented to the pediatric ward during the outbreak. Ninety were confirmed as meningitis cases. The W135 strain of Neisseria meningitidis was responsible for 89 (98.9%) of meningitis cases seen with an incidence rate of 74.9/100,000 in children (0-14 years) and in-hospital case fatality rate of 7.9%. Highest attack rate was among the 12-49 months age group. Clinical features such as meningeal signs (neck stiffness), conjunctivitis, and joint swelling were seen more in cases than controls. Contact history with relatives, who had fever in previous 2 weeks prior to illness was significantly seen more in cases. Adjusted regression analysis showed 7.5 more likelihood of infection with positive contact history (odds ratio [OR]: 7.2 confidence interval [CI]: [3.39-15.73]). There was no significant difference in death outcome between cases and controls (OR: 0.78 CI: [0.29-2.13]). The double peak wave-like pattern of the epidemic curve noted during this outbreak suggests a disseminated outbreak originating from an index case with propagated spread. CONCLUSION: There is need for more effective surveillance and incorporation of vaccine against meningitis into the expanded program on immunization schedule of the Gambia and other countries within the meningitic epidemic belt.

CNS involvement at the onset of primary hemophagocytic lymphohistiocytosis.

OBJECTIVES: To differentiate onset of CNS involvement in primary hemophagocytic lymphohistiocytosis (HLH) from that of other CNS inflammatory diseases and to identify early symptoms linked to abnormal cognitive outcome. METHODS: Forty-six children with primary HLH who had neurologic evaluation within 2 weeks and brain MRI within 6 months of diagnosis were included. Initial symptoms, CSF study, brain MRI, and neurologic outcome were assessed. Brain MRIs were compared with those of 44 children with acute disseminated encephalomyelitis (ADEM). RESULTS: At disease onset, 29 children (63%) had neurologic symptoms and 7 (15%) had microcephaly. Twenty-three (50%) children had abnormal CSF study, but only 15 (33%) had abnormal brain MRI. The latter showed that patients with HLH, unlike patients with ADEM, had symmetric periventricular lesions, without thalamic and brainstem involvement and with infrequent hyposignal intensity on T1. At the end of follow-up (3.6 ± 3.6 years), 17 of the 28 (61%) surviving patients had normal neurologic status, 5 (18%) had a severe neurologic outcome, and 6 (21%) had mild cognitive difficulties. Abnormal neurologic outcome was not influenced by age or type of genetic defect, but by the presence of neurologic symptoms, MRI lesions, or abnormal CSF study at onset. Early clinical and MRI symptoms may regress after treatment. CONCLUSION: Neurologic symptoms are frequent at the onset of primary HLH and are mostly associated with abnormal CSF findings, but with normal brain MRI. In cases of abnormal brain MRI, the observed lesions differ from those of ADEM.

Primary malignant meningeal melanomatosis: a clinical, radiological and pathologic case study.

A 40 year-old woman with subacute headache and visual impairment was admitted. Neurological examination revealed meningismus, diminished visual acuity, bilateral sixth cranial nerve palsy, and papillary edema. Dermatologic examination was normal. The brain CT scan showed hydrocephalus and hyperdense edging around fissures and sulci. The CSF study showed an increased protein level, with persistently negative microbiologic and cytological studies. Prior to Gd-DPTA injection, the brain T1-WI MRI revealed leptomeningeal hyperintensity. A dark subpial substance became evident at cerebral biopsy. The histopatological diagnosis was diffuse leptomeningeal melanomatosis. This case report highlights the diagnostic value of the brain MRI findings in primary leptomeningeal melanomatosis, a rare pathologic condition diagnosed in most published cases only after necropsy. Meningeal T1-WI hyperintensity prior to contrast injection is not caused by sarcoidosis or meningeal carcinomatosis, lymphomatosis or gliomatosis.

Meningismus associated with malpositioned intraspinal catheter for drug delivery.

BACKGROUND: Implanted delivery systems for intrathecal drug administration have become more common in the management of nonmalignant pain. Many postprocedural complications have been described in the literature including infection and headache provoked by position changes. Determining the etiology of a postimplant headache is important particularly when considering the possibility of a life-threatening infection. CASE REPORT: We present a patient who underwent placement of an implantable drug delivery system (IDDS) for intractable abdominal pain that developed positional headaches, and significant neck and back pain. Attempted cerebrospinal fluid aspiration reproduced her symptoms and imaging revealed a malpositioned intraspinal catheter tip approximated to the meninges. Revision of the system completely relieved her symptoms. CONCLUSION: Meningismus from malpositioned catheters is a rare complication that can mimic meningitis but should be considered in the differential for postimplant headaches. Given the increased use of IDDS, it is important to recognize and evaluate postimplant complication and treat it appropriately. We discuss this case report and appropriate work-up and differential diagnosis for meningismus following implant.

[When should a patient with headaches be referred to the emergency ward?]. / Quand référer aux urgences un patient présentant une céphalée?

Secondary headaches are rare though potentially severe. A systematic search of red flags helps to suspect headaches of secondary origin that require further urgent investigation. Main red flags are: sudden onset, exceptionally severe headache, new headache in patient over 50, vomiting or syncope, focal neurological sign or neck stiffness, recent trauma, uncommon headache during pregnancy or anticoagulant therapy, suspicion of glaucoma.