Case Reports / Journal of Clinical Neuroscience 21 (2014) 683–685 our understanding of CAPS and to develop consensus-based treatment recommendations. Conflicts of interest/disclosure The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication. References [1] Dafer R, Biller J. Antiphospholipid syndrome: role of antiphospholipid antibodies in neurology. Hematol Oncol Clin North Am 2008;22:95–105. [2] Merrill J, Asherson R. Catastrophic antiphospholipid syndrome. Nat Clin Pract Rheumatol 2006;2:81–6. [3] Cervera R. Update on the diagnosis, treatment, and prognosis of the catastrophic antiphospholipid syndrome. Curr Rheumatol Rep 2010;12:70–6. [4] Kirollos R, Tyagi AK, Rossa SA, et al. Management of spontaneous cerebellar hematomas: a prospective treatment protocol. Neurosurgery 2001;49:1378–87. [5] Amar AP. Controversies in the neurosurgical management of cerebellar hemorrhage and infarction. Neurosurg Focus 2012;32:E1. 683 [6] Wu YT, Li TY, Lu SC, et al. Predictors of first-week mortality in patients with acute spontaneous cerebellar hemorrhage. Cerebellum 2013;12:165–70. [7] Arinuma Y, Kikuchi H, Aramaki K, et al. Histopathological analysis of cerebral hemorrhage in systemic lupus erythematosus complicated with antiphospholipid syndrome. Mod Rheumatol 2011;21:509–13. [8] Cervera R, Font J, Gomez-Puerta JA, et al. Validation of the preliminary criteria for the classification of catastrophic antiphospholipid syndrome. Ann Rheum Dis 2005;64:1205–9. [9] Finis A, Ssenyonjo H, Knopp U, et al. Infarction of the right hemisphere in a patient with antiphospholipid antibody syndrome. Acta Neurochir (Wien) 2005;147:997–1002. [10] Inoue R, Katayama S, Kasai N, et al. Middle cerebral artery occlusion with unilateral moyamoya like vessels and with ruptured anterior cerebral artery aneurysm–its relation to the antiphospholipid antibody syndrome. No To Shinkei 1994;46:995–8. [11] Miesbach W, Scharrer I, Asherson RA. Recurrent life-threatening thromboembolism and catastrophic antiphospholipid syndrome in a patient despite sufficient oral anticoagulation. Clin Rheumatol 2004;23:256–61. [12] Nagai S, Horie Y, Akai T, et al. Superior sagittal sinus thrombosis associated with primary antiphospholipid syndrome–case report. Neurol Med Chir (Tokyo) 1998;38:34–9. [13] Sakamoto S, Akutsu K, Kawase K, et al. Simultaneous presentations of deep vein thrombosis and cerebral sinus thrombosis in a case of primary antiphospholipid syndrome. Angiology 2008;59:765–8. http://dx.doi.org/10.1016/j.jocn.2013.05.015 Acute inferior homonymous quandrantanopia in a 71-year-old woman Ivana Vachalová, Viola Gindl, Josef G. Heckmann ⇑ Department of Neurology, Municipal Hospital Landshut, Robert-Koch Str. 1, Landshut 84034, Germany a r t i c l e i n f o Article history: Received 4 April 2013 Accepted 15 May 2013 Keywords: Creutzfeldt–Jakob disease Heidenhain variant Hemianopia Quadrantanopia Stroke mimics a b s t r a c t A 71-year-old woman presented with acute inferior homonymous quadrantanopia initially mimicking acute ischemic stroke. As clinical signs and symptoms progressed to akinetic mutism with myoclonus the diagnosis of the Heidenhain variant of Creutzfeldt–Jakob disease was made. Brain MRI 4 days after symptom onset revealed ribbon-like high signal intensity in the medial occipital cortex. Ó 2013 Elsevier Ltd. All rights reserved. 1. Introduction Homonymous hemianopic disorders are commonly encountered in neurological practice and need a careful diagnostic work-up [1]. Stroke is one major cause of retrochiasmal dysfunction, but can be mimicked by a number of other neurological conditions [2]. Here we describe a patient with the Heidenhain variant of Creutzfeldt–Jakob disease (CJD) initially mimicking acute stroke with sudden onset of quadrantanopia. 2. Case report A 71-year-old woman with a history of hypertension reported acute visual disturbances on the right side in the lower visual field and consulted her ophthalmologist. The ophthalmological exploration revealed a visual acuity of 0.3 in the right eye and 0.7 in the left eye. An amblyopia and divergent strabismus had been known since childhood. Ocular tension was normal (16 mmHg bilaterally) ⇑ Corresponding author. Tel.: +49 871 698 3719. E-mail address: josef.heckmann@klinikum-landshut.de (J.G. Heckmann). and a fundoscopic exploration revealed slight hypertonic changes. The exploration of the visual fields revealed an inferior homonymous quandrantanopia on the right side (Fig. 1a) prompting MRI, which indicated hyperintense signal in the left medial occipital cortex (Fig. 1b). Combined with the acute onset and circumscribed symptomatology, an ischemic stroke in the territory of the posterior cerebral artery was suspected. As the vascular risk factor of arterial hypertension was known and atrial fibrillation was newly detected, an oral anticoagulant and intensification of the antihypertensive therapy were administered. Some weeks later, the patient’s status deteriorated rapidly: she became blind and mute, and a myoclonic movement disorder developed necessitating in-hospital care. At this time, electroencephalography detected general slowing and periodic triphasic waves (Fig. 1c). A second MRI revealed parieto-occipital progress of the cortical hyperintensities (Fig. 1d) and, in addition, changes in the basal ganglia and the thalamus. Protein 14-3-3 was elevated in the cerebrospinal fluid. A detailed medical history from relatives gave no history of an earlier dura mater graft or a corneal transplant [3]. The combination of the rapidly developing clinical status, history, and paraclinical findings led to the diagnosis of the Heidenhain variant of CJD, and palliative care was implemented. 684 Case Reports / Journal of Clinical Neuroscience 21 (2014) 683–685 Fig. 1. (a) Visual field test of the (LA) left eye and (RA) right eye showing inferior homonymous quadrantanopia on the right side. (b) Axial diffusion-weighted brain MRI taken 4 days after detection of the inferior quadrantanopia showing hypertintense, ribbon-shaped signal irregularities predominately in the medial visual cortex (white arrows). (c) Electroencephalography showing general slowing with triphasic waves (grey arrow). (d) Second axial diffusion-weighted MRI following clinical deterioration showing progression of the parieto-occipital cortical hyperintensities predominately in the left hemisphere (white arrows). 3. Discussion The Heidenhain variant of CJD was first reported in 1954, and the original description of Heidenhain goes back to 1929 [4]. The Heidenhain variant is a clinical term that describes prominent visual disturbances such as hemianopia, cortical blindness, optical hallucinations, palinopsia, or metachromatopsia. It is reported in 3.7–20% of CJD cases, which occur at an incidence of 1 case per 1 million people per year [4,5]. Diagnosis of CJD at its clinical manifestation is intricate, and misdiag- noses at this time are not uncommon. In particular, some patients have recently been reported to mimic acute stroke, such as our patient [6–9]. Whether the Heidenhain variant constitutes a real, distinct type of CJD cannot currently be determined. However, it is characterized by some different features. In addition to the prominent visual symptoms at the beginning, the course of the disease is shorter, the neuropathological changes are more likely to be localized in the posterior brain regions, and the MRI findings tend to be located in the parieto-occipital area of the brain. In addition, homozygosity Case Reports / Journal of Clinical Neuroscience 21 (2014) 685–688 for methionine at codon 129 of the prion protein is more frequent, which, however, was not tested in our patient [5]. In conclusion, from our patient we have learned two lessons. First, prion disease can manifest in an acute manner with signs and symptoms mimicking stroke. Thus, in day-to-day clinical practice, this differential diagnosis has to be considered. Because visual symptoms can initially predominate, ophthalmologists should also be aware of this clinical condition, particularly if they plan invasive procedures [4,10]. However, over-diagnosis is also a risk and should be avoided [11]. Second, 4 days after the onset of clinical symptoms, an MRI revealed an abnormality which retrospectively was recognised to be a nearly pathognomonic pattern, which would have allowed an early diagnosis. In the literature, a positive early MRI has been reported during the 9 days after symptom onset [12]. Unfortunately, this finding was misinterpreted in our patient as it was probably confounded by the history of acute onset and circumscribed symptomatology. Recently, MRI has been proposed to be included in the diagnostic criteria for CJD because the pattern of hyperintensity and restricted diffusion can differentiate CJD from other forms of rapid dementia with high sensitivity and specificity [13]. However, interpretation requires thorough training of radiologists, as well as neurologists, in order to become familiar with these findings. Conflicts of interest/disclosure The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication. Acknowledgments The authors thank Prof. Dr. Inga Zerr (Prion Research Institute, Göttingen, Germany) for analysis of protein 14-3-3 and expert discussion of the clinical findings; Dr. Kretz (Ophthalmologist’s Office 685 Landshut, Germany) for providing the ophthalmological results; the Radiological Institute Mühleninsel (Landshut, Germany) for providing the first MRI findings, and PD Dr. Dinkel (Radiological Department, Municipal Hospital, Landshut, Germany) for providing the second MRI findings. References [1] Fraser JA, Newman NJ, Biousse V. Disorders of the optic tract, radiation, and occipital lobe. Handb Clin Neurol 2011;102:205–21. [2] Heckmann JG, Stadter M, Dütsch M, et al. Hospitalization of nonstroke patients in a Stroke Unit. Dtsch Med Wochenschr 2004;129: 731–5. [3] Lang CJ, Heckmann JG, Neundörfer B. Creutzfeldt-Jakob disease via dural and corneal transplants. J Neurol Sci 1998;160:128–39. [4] Cooper SA, Murray KL, Heath CA, et al. Isolated visual symptoms at onset in sporadic Creutzfeldt-Jakob disease: the clinical phenotype of the ‘‘Heidenhain variant’’. Br J Ophthalmol 2005;89:1341–2. [5] Kropp S, Schulz-Schaeffler WJ, Finkenstaedt M, et al. The Heidenhain variant of Creutzfeldt-Jakob disease. Arch Neurol 1999;56:55–61. [6] Hohler AD, Flynn FG. Onset of Creutzfeldt-Jakob Disease mimicking an acute cerebrovascular event. Neurology 2006;67:538–9. [7] Szabo K, Achtnichts L, Grips E, et al. Stroke-like presentation of CreutzfeldtJakob disease. Cerebovasc Dis 2004;18:251–3. [8] Lyytinen J, Sairanen T, Valanne L, et al. Progressive stroke-like symptoms in a patient with sporadic Creutzfeldt-Jakob disease. Case Rep Neurol 2010;2:12–8. [9] Hirst CL. Sporadic Creutzfeldt-Jakob-Disease presenting as a stroke mimic. Br J Hosp Med (Lond) 2011;72:590–1. [10] Amstrong RA. Creutzfeldt-Jakob disease and vision. Clin Exp Optom 2006;89:3–9. [11] Chitravas N, Jung RS, Kofskey DM, et al. Treatable neurological disorders misdiagnosed as Creutzfeldt-Jakob disease. Ann Neurol 2011;70:437–44. [12] Bekiesińska-Figatowska M, Kuczyńska-Zardzewiały A, Pomianowska B, et al. The value of magnetic resonance imaging in the early diagnosis of CreutzfeldtJakob disease – own experience. Pol J Radiol 2012;77:63–7. [13] Vitali P, Maccagnano E, Caverzasi E, et al. Diffusion-weighted MRI hyperintensity patterns differentiate CJD from other rapid dementias. Neurology 2011;76:1711–9. http://dx.doi.org/10.1016/j.jocn.2013.05.015 Vertebral artery dissection after neck extension in an adult patient with Klippel–Feil syndrome David Dornbos III a,⇑, Daniel S. Ikeda a, Andrew Slivka b, Ciaran Powers a a b Ohio State University Wexner Medical Center, Department of Neurosurgery, N1014 Doan Hall, 410 West 10th Avenue, Columbus, OH 43210, USA Ohio State University Wexner Medical Center, Department of Neurology, Columbus, OH, USA a r t i c l e i n f o Article history: Received 26 June 2013 Accepted 12 July 2013 Keywords: Cervical hypermobility Klippel–Feil syndrome Vertebral artery dissection a b s t r a c t The association between Klippel–Feil syndrome and vertebral artery dissection is quite rare. We report an adult patient with vertebral artery dissection and Klippel–Feil syndrome, to our knowledge only the third reported case of its kind. A 45-year-old woman with a known history of Klippel–Feil syndrome presented with occipital head and neck pain following forced neck extension. Diagnostic cerebral angiography revealed a high grade vertebral artery stenosis, consistent with vertebral artery dissection. Following 6 months of medical management, a repeat diagnostic angiogram revealed complete healing of the vessel. While cervical fusion, as seen in Klippel–Feil syndrome, has previously been shown to cause neurologic injury secondary to hypermobility, the association with vertebral artery dissection is incredibly rare. We hypothesize that this hypermobility places abnormal shear force on the vessel, causing intimal injury and dissection. Patients with seemingly spontaneous vertebral artery dissection may benefit from cervical spine radiography, and this predisposition to cerebrovascular injury strongly suggests further evaluation of vascular injury following trauma in patients with Klippel–Feil syndrome or other cervical fusion as clinically warranted. Ó 2013 Elsevier Ltd. All rights reserved. ⇑ Corresponding author. Tel.: +1 614 293 0821; fax: +1 614 293 4281. E-mail address: David.dornbos@osumc.edu (D. Dornbos III).