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Background: The "hot cross bun" sign is a cruciform hyperintensity is seen on T2 weighted imaging within the pons. The sign is considered to be pathognomic for Multiple system atrophy type C. The clinical and radiological features of Multiple system atrophy type C overlap with the autosomal dominant inherited ataxias. We present a case series of 3 African patients with genetically proven Spinocerebellar Ataxia presenting with the Hot cross bun sign and a scoping review of similar studies. Cases: We described the phenotypic and radiological presentation of genetically confirmed SCA-2 in two, and SCA-7 in one patient, with the "hot cross bun" sign. Literature Review: We performed a scoping review on the Hot Cross Bun Sign.A total of 66 articles were retrieved. We describe the diverse aetiologies of the sign and associated phenotypic and radiological features. We review the Spinocerebellar Ataxias described with a Hot cross bun sign and make comparisons to Multiple System Atrophy Type C [Ref. 1,2]. Conclusions: To our knowledge this is the first description of an African cohort presenting with the Hot Cross Bun Sign. We expand the differential diagnosis of the Hot Cross Bun Sign.
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INTRODUCTION: The underlying diagnosis of cavernous sinus disease is difficult to confirm in HIV-coinfected patients owing to the lack of histological confirmation. In this retrospective case series, we highlight the challenges in confirming the diagnosis and managing these patients. RESULTS: The clinical, laboratory and radiological data of 23 HIV-infected patients with cavernous sinus disease were analysed. The mean age of patients was 38 years. The mean CD4+ count was 390 cells/µL. Clinically, patients presented with unilateral disease (65%), headache (48%), diplopia (30%) and blurred vision (30%). Third (65%) and sixth (57%) nerve palsies in isolation and combination (39%) were most common. Isolated fourth nerve palsy did not occur. Tuberculosis (17%) was the most commonly identified disorder followed by high-grade B-cell lymphoma (13%), meningioma (13%), metastatic carcinoma (13%) and neurosyphilis (7%). In 22% of the patients, there was no confirmatory evidence for a diagnosis. The patients were either treated empirically for tuberculosis or improved spontaneously when antiretroviral therapy was started. Cerebrospinal fluid was helpful in 4/13 (31%) of patients where it was not contraindicated. Only 3/23 (13%) of the patients had a biopsy of the cavernous sinus mass. The outcomes varied, and follow-up was lacking in the majority of patients. CONCLUSION: In HIV-infected patients, histological confirmation of cavernous sinus pathology is not readily available for various reasons. In resource-limited settings, one should first actively search for extracranial evidence of tuberculosis, lymphoma, syphilis and primary malignancy and manage appropriately. Only if such evidence is lacking should a referral for biopsy be considered.
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BACKGROUND: The association of the anti-aquaporin-4 (AQP-4) water channel antibody with neuromyelitis optica (NMO) syndrome has been described from various parts of the world. There has been no large study describing this association from southern Africa, an HIV endemic area. HIV patients often present with visual disturbance or features of a myelopathy but seldom both either simultaneously or consecutively. We report our experience of NMO in the era of AQP-4 testing in HIV-positive and HIV-negative patients seen in KwaZulu-Natal, South Africa. METHODS: A retrospective chart review was undertaken of NMO cases seen from January 2005 to April 2016 in two neurology units serving a population of 7.1 million adults. The clinical, radiological and relevant laboratory data were extracted from the files and analysed. RESULTS: There were 12 HIV-positive patients (mean age 33 years), 9 (75%) were women and all 12 were black patients. Of the 17 HIV-negative patients (mean age 32 years), 15 (88%) were women and 10 (59%) were black people. The clinical features in the two groups ranged from isolated optic neuritis, isolated longitudinally extensive myelitis or combinations. Recurrent attacks were noted in six HIV-positive patients and six HIV-negative patients. The AQP-4 antibody was positive in 4/10 (40%) HIV-positive patients and 11/13 (85%) HIV-negative patients. The radiological changes ranged from longitudinal hyperintense spinal cord lesions and long segment enhancing lesions of the optic nerves. Three patients, all HIV-positive, had tumefactive lesions with incomplete ring enhancement. CONCLUSION: This study confirms the presence of AQP-4-positive NMO in southern Africa in both HIV-positive and HIV-negative patients. The simultaneous or consecutive occurrence of optic neuritis and myelitis in an HIV-positive patient should alert the clinician to test for the AQP-4 antibody. It is important to recognise this clinical syndrome as specific therapy is available. We further postulate that HIV itself may act as a trigger for an autoimmune process.
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The relationship between pseudotumor cerebri and contraceptive drugs is controversial. Its association with Implanon, an implantable single-rod contraceptive containing etonogestrel (a progestogen) has not been reported but is the subject of many medico-legal cases. The authors present two case reports of patients using Implanon and who subsequently developed pseudotumor cerebri. Rapid weight gain rather than direct hormonal influence is probably the trigger. Headaches, visual obscurations, and rapid weight gain in patients using Implanon should alert one to the probable diagnosis of pseudotumor cerebri.
