Case Report Facing up to a problem with recognition In 1891 Sigmund Freud introduced the term agnosia, meaning an absence of knowledge, to describe deficits of higher sensory processing which cause impaired recognition. These disorders are of great interest to neuropsychologists who try to characterize brain–behaviour interrelationships. Agnosias may be perplexing for both patients and clinicians, prompting an initial and understandable consideration of a primary sensory deficit (McCormick and Larner, 2018). Agnosias may occur in any sensory modality but those affecting the visual domain are the most commonly encountered in clinical practice (Farah, 1995). Visual agnosias may result in impaired recognition of objects or of the written word, the latter manifesting as an acquired inability to read (alexia). More circumscribed visual agnosias may also occur, for example restricted to colours. This article reports a patient who presented with a sudden onset of inability to recognize familiar faces. Discussion Prosopagnosia is a rare, circumscribed form of visual agnosia characterized by an inability to recognize previously known human faces or equivalent stimuli (Farah, 1995; Mayer and Rossion, 2007; Rivolta, 2014; Corrow et al, 2016; Larner, 2016). The defect is not necessarily limited solely to faces; it may encompass other categories such as animals (‘zooagnosia’; Assal et al, 1984; Larner, 2016). Dr S McCrory, Specialist Registrar in Neurology, Walton Centre for Neurology and Neurosurgery, Liverpool Dr DF Smith, Consultant Neurologist, Walton Centre for Neurology and Neurosurgery, Liverpool Dr AJ Larner, Consultant Neurologist, Walton Centre for Neurology and Neurosurgery, Liverpool L9 7LJ Correspondence to: Dr AJ Larner (a.larner@thewaltoncentre.nhs.uk) 288 Prosopagnosia is associated with lesions in the right inferior occipito-temporal region of the brain, particularly involving the lingual and fusiform gyri and subjacent white matter. This cerebral localization means that the disorder of facial recognition may be associated with other neurological features such as visual field defect (left upper quadrantanopia or homonymous hemianopia, although for the diagnosis of prosopagnosia to be made this should not be sufficient to produce a perceptual deficit) and/or achromatopsia, neither of which was evident in this patient. However, she did complain of visual distortions (‘long noses’) suggestive of metamorphopsia, which has on occasion been associated with prosopagnosia (e.g. Seron et al, 1995). The aetiology of prosopagnosia is broad. It most often results from stroke (infarct or haemorrhage) but has also been reported on occasion in association with carbon monoxide poisoning, temporal lobectomy, encephalitis, neoplasm, trauma, and with Parkinson’s disease and Alzheimer’s disease (Mayer and Rossion, 2007). A form of frontotemporal dementia with focal right temporal atrophy may present with progressive prosopagnosia (Evans et al, 1995). A developmental or congenital form of prosopagnosia has also been described (e.g. Larner et al, 2003) and these cases have been increasingly studied in recent years to try to understand the underlying neuropsychological deficit responsible for the syndrome. To the authors’ knowledge there is only one previous report of prosopagnosia in association with cerebral amyloid angiopathy (Hainline et al, 2017), and this appears to have been an inflammatory variant of this condition. Prosopagnosia is sometimes characterized as ‘face blindness’ but this terminology may be a misnomer since patients are not blind CASE REPORT A 63-year-old right-handed woman presented with a new onset of visual problems, complaining of an inability to recognize faces. This had developed suddenly, about 3 months before her presentation to the neurology clinic. She was unable to discern family members or familiar TV characters by their face alone, although she reported that she could recognize them based on other cues such as the sound of their voices, general body habitus, movement and clothing. She said that faces appeared grey and blank with odd ‘long noses’. Because of the visual symptoms she had attended an ophthalmology outpatient clinic, but no defect was found in visual acuity or on field testing or ophthalmoscopy. Her reading ability was unimpaired. Her past medical history included a transient ischaemic attack and hypercholesterolaemia, treated with aspirin and atorvastatin respectively. She was an ex-smoker (25 pack years). There was no family history of similar visual problems or stroke. Neurological examination disclosed no focal abnormalities, in particular there was no visual field defect or impairment of colour vision. Magnetic resonance imaging of the brain was performed. On standard sequences (not shown) there were some patchy punctate high signal lesions in the cortical white matter typical of small vessel ischaemia. However, on susceptibility weighted imaging, a technique of particular value in detecting blood products which distort the local magnetic field (Cheng et al, 2013), diagnostic changes were seen (Figure 1). There was a circumscribed area of signal change in the right inferior occipito-temporal region, indicative of a previous haemorrhage. In addition, there were a number of small dot-like areas of signal change, indicative of microbleeds. A diagnosis of acute-onset prosopagnosia as a consequence of a right inferior occipitotemporal region cerebral haemorrhage was made. The combination of lobar haemorrhage and microbleeds indicated an underlying pathological diagnosis of cerebral amyloid angiopathy. At follow up the patient continued to complain of difficulty discriminating faces in detail. © 2019 MA Healthcare Ltd Introduction British Journal of Hospital Medicine, May 2019, Vol 80, No 5 Downloaded from magonlinelibrary.com by 130.237.122.245 on September 7, 2019. Case Report Figure 1. Axial susceptibility-weighted magnetic resonance brain imaging showing (a) a circumscribed area of signal change in the right inferior occipito-temporal region indicative of a previous haemorrhage, with (a, b) a number of separate, dot-like areas of signal change indicative of microbleeds, the combination suggesting an underlying pathological diagnosis of cerebral amyloid angiopathy. a LEARNING POINTS ■■ A deficit in the ability to recognize familiar faces in the absence of significant impairment of visual acuity or visual fields has been termed prosopagnosia. b ■■ Prosopagnosia is rare, but the symptoms are characteristic. ■■ Prosopagnosia most commonly results from stroke (infarct or haemorrhage) affecting the right inferior occipitotemporal region. ■■ A developmental or congenital form of prosopagnosia is also described. ■■ Attempted rehabilitation has little to offer, but patients can often use other visual and non-visual cues to achieve recognition. Forthcoming case reports Case Report A case of flash pulmonary oedema secondary to preeclampsia at 33 weeks’ gestation Introduction Rectourethral fistula – the result of an unusual sequence of events Delayed presentation of neonatal clavicle fracture – a management challenge Persistent headache: a case of post-traumatic cerebral venous thrombosis Acute A feverish junior doctor interstitial nephritis caused by two differ misse d n pump inhibitors proto with a diagnosis not to beent (2013) report a case of Adewole et alnephritis, presentation years with a further clinical ovale) result inAcute P. ovale neverepisode of acute approved the sale of proton interstitial nephritis in a man who had presenting pump inhibitors P. vivax important interstitial endemic setting. is an nephritis when rechallenged ‘over from the after returning cause of acute kidney injurytraveled the counter’. The increased availability an area and so clinicians to suchwith and drugs a different proton attributed to hypnozoite This has been account for tropical diseasespump inhibitor of these medications has lead to improved for about remain vigilant cases. Since liverof should in the85% (pantoprazole). Previous As forms of the parasite 1992 itresiding has beenyears non-endemic setting. case studies have awareness and documentation of the sideestablished that protonin the later presenting shown that reintroduction of the same effect profile. While proton and persisting until Shingadia, pumpreactivation and inhibitors can authors (Ladhani reported cause acute pump inhibitorpreviously interstitial proton pump inhibitor In light of this, the caused recurrent induced acute interstitial (Krotoski, 1985). nephritis. al, 2013), delayed or missed Mant et worsening nephritis 2011; is a rare travel overseas all of about renal function. advocate enquiringThis case report describes but idiosyncratic reaction, the prevalence can result in death, of malaria 47-year-old of This is the first report and years, rather than just the a diagnosis acute interstitial proton pump inhibitor within the last 3–5 woman who presented accurate travel history, of with acute prescribing makes it by eliciting kidney annephritis taught, to prevent occurring secondary to two an as currently last 12 months injury increasing problem (Sierra et al, 2007). secondary to proton pumpremaining identifying rarer diseases inhibitor-vigilant, different proton pump inhibitors being overlooked. important diagnoses or in the same (omeprazole) Proton pump inhibitor-induced acute dengue, enteric fever induced acute such as malaria, interstitial individual. This strengthens During 2014, there were just 1586 cases the hypothesis easier, interstitial nephritis has been well described becomes much rickettsial infections that proton pump inhibitor-induced acute in many of imported malaria in the UK (Health (2012). series Bell by and four highlighted case as reports clinical Dr Frederick have so is interstitial nephritis is a class effect and Torlot 2015),JA Foundation Year 2 Protection Agency, and shown that the renal function Doctor in the units deteriorates Department is limited, should raise caution when clinicians consider of of specialist experience outsideGastroenterology on reintroduction of the same proton and Hepatology Conclusions Discussion initiating another proton the inhibitor of the non-specific which makes recognition in pump inhibitor (Christensen et Dr Duncan J Whitehead authors’ experience andpump Given This case highlights the importance of taking al, 1993; is Consultant Acute the a patient who has challenging. The number of diagnosed Badov et al, 1997; Landray in features of malaria the value been Physician and Nephrologist in cthe changing malarial epidemiology,previously a thorough and extended travel history et al, 1998; with proton pump inhibitor-induce to malaria-endemi Department d acute Ra and Tobe, 2004). This -like of people travelling of Acute Internal Medicine film in situations of clinical and a blood article presents any patient presenting with a fever or flu-like (United requesting interstitial to increase nephritis.information Nephrology, continues Musgrove countries Park such Hospital, the species further first For case of proton pump inhibitoruncertainty is obvious. symptoms. Certain malarial 2012). Taunton Organization, and Somerset NHS Foundation Nations World Tourism induced acute interstitial nephritis caused as Plasmodium ovale can have incubation Trust, Taunton TA1 5DA Discussion by two different proton pump inhibitors. periods of several years, which is important Correspondence to: Dr FJA Torlot Proton pump inhibitors are one of the most This is important because it (f.torlot@gmail.com) demonstrates for health-care professionals to appreciate. commonly prescribed drug classes and, of in that proton pump inhibitor-induce Case RepORT Mellon et al (2014) describe two cases d acute 2003, the Food countDrug of Administration after thrombocytopenia with a platelet and interstitial nephritis is a class effect, despite 9 P. ovale presenting 47 and 69 months A previously healthy 24-year-old Caucasian 9/litre (normal 150–400×10 /litre) and 32×10 study initial exposure. A large observational junior doctor presented to an accident and 177 IU/ raised alanine aminotransferase level of found with a 5-day history emergency department conducted by Broderick et al (2015) and urine CAse RepoRT litre (normal 3–35 IU/litre). Chest X-ray to the of drenching night sweats associated with serology HIV that of all the malaria cases imported unremarkable. both were dipstick generally was and feeling A 47-year-old woman a severe headache proved to was referred to the UK since 1987, the commonest species were subsequently both stopped. and blood cultures She had a renal biopsy 4 days profound of complained vivax medical P. patient by The assessment departments unwell. unit with a 5-monthbehistory and a review of the literature P. falciparum (65.5%), followed negative.later, which showed acute interstitial nephritis of vomiting episodedry onemalaise, of general regarding proton pump inhibitor-induced rigors and reported blood cough and anorexia. The bloodwith (3.5 ml of venous filmeosinophilic acute (25.4%), P. ovale (6.0%) and P. malariae nephritis and granulomatous She hours. had Past beenmedical interstitial nephritis, it was agreed that found tohistory in the last 24 have an elevated on both thickinfiltration, as the National and thin films the (1.6%). Species identification is achieved features suggestive of a drugwas not and the patient serum creatinine patient should be tried on pantoprazole. was unremarkable level by (2010) initial her GP and referred Care Excellence Health andaetiology. The Institute for mediated by conducting a ‘thin film’ after The culprit A 1-year medications. to theany acute potential for a relapse of the acute interstitial medical take. currently taking Her past on admissiondrug was film’. suspected totaken guidelines recommend) be omeprazole, trip to medical identification of malaria from a ‘thick 3-week which she had of atype history consisted included nephritis was explained to the patient. She travel history 2 diabetes mellitus, is shown inbeen this unexpectedly 1 andregularly the Figure taking for the previous 7 years, Francisco. Broderick et al (2015) reported that visit to San gastro-oesophagea a 10-day andBarrett’s oesophagus, proceeded Greece ovale, with weekly Plasmodium blood tests to monitor lrevealed ansoinfection (like this waswith stopped. Naproxen had not been drug use but did any illicit variable latency period of P. vivax can denied disease, her serum creatinine level and over The patientreflux musculoskeletal back pain and of protozoa responsible for The differentials for a febrile patient illness presenting with a non-specific flu-like are broad and far-reaching, but eliciting is an accurate and extensive travel history a crucial first step in reaching a diagnosis. cases This is particularly important in tropical endemic the presenting outside of disease setting. This case report illustrates will the aforementioned learning point and hopefully decrease the likelihood of a delayed or missed diagnosis. Dr Oliver Johnson is Academic Foundation the Year 1 in Diabetes and Endocrinology in Department of Diabetes and Endocrinology, Queen Elizabeth Hospital, Birmingham B15 2GW Doctor Dr David Walker is Foundation Year 2 in the Acute Medical Unit, Royal Bournemouth Hospital, Bournemouth in Dr Tom Cummin is Specialist Registrar Haematology in the Department of Haematology, Royal Bournemouth Hospital, Artery of Percheron infarction – a rare stroke case report Decompressive talc pleurodesis for hepatic hydrothorax Bournemouth Correspondence to: Dr O Johnson (droliverjohnson@gmail.com) Recurrent hypoglycaemia and cognitive impairment: a 14-year follow-up British Journal of Hospital Medicine, May 2019, Vol 80, No 5 Case Report Introduction Anti-LGI1 autoimmune encephalitis: a treatable condition presenting with subacute cognitive decline and hyponatraemia © 2019 MA Healthcare Ltd Doran M. Developmental prosopagnosia: a clinical and neuropsychological study. J Neurol. 2003;250(Suppl2): II156 (abstract P591) Livingston LA, Shah P. People with and without prosopagnosia have insight into their face recognition ability. Q J Exp Psychol. 2018 May;71(5):1260–1262. https://doi.org/10.1080/1 7470218.2017.1310911 Mayer E, Rossion B. 2007. Prosopagnosia. In: Godefroy O, Bogousslavsky J, eds. The behavioural and cognitive neurology of stroke. Cambridge: Cambridge University Press: 315–334 McCormick LJ, Larner AJ. ‘Could you repeat that?’: not always a hearing problem. Br J Hosp Med. 2018 Jun 02;79(6):350–351. https://doi. org/10.12968/hmed.2018.79.6.350 Rivolta D. 2014. Prosopagnosia. When all faces look the same. Berlin: Springer. https://doi.org/ 10.1007/978-3-642-40784-0 Seron X, Mataigne F, Coyette F, Rectem D, Bruyer R, Laterre EC. Etude d’un cas de métamorphopsie limitée aux visages et à certains objets familiers. Rev Neurol (Paris). 1995 Dec;151(12):691–698. types taken one of several in the previous 3–4 months and before 2 weeks sexual hypertension. Herintercourse report unprotected medications on admission that infrequently. causing malaria. of HIV the possibility which raised naproxen, previouslyincluded omeprazole and paracetamol. history into the travel Further probing Oral prednisolone 60 mg and ranitidine evidence of a was no was ClinicalThere examination seroconversion. unremarkable andrevealed awere and Swaziland Mozambique trip tostarted. Her gastro-oesophageal reflux Review of or neck stiffness. she was euvolaemic. rash, photophobia Her serum creatinine 18 during the patient’s level previouslysignificantly monthssymptoms worsened, negative. 202 umol/litre systems was otherwise at presentation; 5 months antimalarial necessitating elective, school prednisolone medical towhen be rapidly weaned and alert and previouslythe herpatient On admission, serumwas creatinine concentration The patient was taken. was not stopped prophylaxis over 2 weeks and tachycardic she was (40.8°C), had pyrexial but been 50 umol/litre. orientated Urine dipstick showed chloroquine and prescribed treated with a domperidone. subsequently During this period her renal tachypnoeic andand trace of blood (145 beats/minute) protein. Ultrasonography primaquine several being discharged of beforeimproved function rapidly; her creatinine level was pressure the kidney,Blood ureters (35 breaths/min). and bladder up. It is worth indicated normal outpatient to 99 days laterfellwith umol/litrefollow 28 days after commencing saturation of oxygen with anand sized kidneys 117/74 mmHg no evidence leukopenia and of an obstructed thethrombocytopenia, prednisolone. noting that air. The history offered no obvious roomsystem. 98% onrenal liver function deranged Despite mild-to-moderately the medication clinical while changes cause of pyrexia, drug-induced clues as to Athe acute interstitial nephritistests, assymptoms are typical of a her reflux patient, seen in this had a major impact on her sleep and splenomegaly. was suspected revealed marked examination as the cause resolved of her acute splenomegaly The infection. malarialsense of wellbeing. After consulting colleagues cell count of a white revealed kidney tests injury. Naproxen Initial blood and omeprazole wereafter a period of 8 weeks. in the gastroenterology 9 and nephrology 2.2×109/litre (normal 4–11×10 /litre), profound 50 46 British Journal of Hospital Medicine, 14 days this had risen to 125 umol/litre, although her reflux symptoms had markedly improved. She was otherwise systemically well at that time, and the clinical diagnosis of recurrent proton pump inhibitor-induced acute interstitial nephritis was made and her pantoprazole was immediately stopped. Subsequently her serum creatinine concentration fell over 3 months to 86 umol/litre, which is now her baseline level (Figure 1). She was started on famotidine in place of ranitidine as a result of poor symptom control some months later. The gastroenterology team have deemed her Barrett’s oesophagus as low risk and she will remain under their surveillance. January 2016, Vol 77, No 1 British Journal of Hospital Medicine, January © 2016 MA Healthcare Ltd Assal G, Favre C, Anderes JP. [Nonrecognition of familiar animals by a farmer. Zooagnosia or prosopagnosia for animals]. Rev Neurol (Paris). 1984;140(10):580–584. Cheng AL, Batool S, McCreary CR, Lauzon ML, Frayne R, Goyal M, Smith EE. Susceptibilityweighted imaging is more reliable than T2*-weighted gradient-recalled echo MRI for detecting microbleeds. Stroke. 2013 Oct 01;44(10):2782–2786. https://doi.org/10.1161/ STROKEAHA.113.002267 Corrow S, Dalrymple K, Barton J. Prosopagnosia: current perspectives. Eye Brain. 2016 Sep;8:165– 175. https://doi.org/10.2147/EB.S92838 Evans JJ, Heggs AJ, Antoun N, Hodges JR. Progressive prosopagnosia associated with selective right temporal lobe atrophy. Brain. 1995 Feb;118(1):1– 13. https://doi.org/10.1093/brain/118.1.1 Farah MJ. 1995. Visual agnosia: disorders of object recognition and what they tell us about normal vision. Cambridge: MIT Press: 104–112 Hainline C, Rucker JC, Zagzag D et al. Tumoral presentation of homonymous hemianopia and prosopagnosia in cerebral amyloid angiopathyrelated inflammation. J Neuroophthalmol. 2017 Mar;37(1):48–52. https://doi.org/10.1097/ WNO.0000000000000474 Larner AJ. 2016. A dictionary of neurological signs. 4th edn. London: Springer. https://doi.org/ 10.1007/978-3-319-29821-4 Larner AJ, Downes JJ, Hanley JR, Tsivilis D, © 2016 MA Healthcare Ltd to all facial features. They may be able to identify emotional expression and eye gaze direction (Larner et al, 2003), and features such as hair or a moustache may be used to aid recognition. Patients with acquired prosopagnosia retain insight into their deficit in face recognition (Livingston and Shah, 2018). Attempted rehabilitation in acquired prosopagnosia has achieved, at best, only modest results (Mayer and Rossion, 2007; Corrow et al, 2016). BJHM 2016, Vol 77, No 1 289 Downloaded from magonlinelibrary.com by 130.237.122.245 on September 7, 2019.