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A Complicated Case of Von Hippel-Lindau Disease

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A Complicated Case of Von Hippel-Lindau Disease Postgrad Med J 2001;77:471–480 471 Postgrad Med J: first published as 10.1136/pmj.77.909.478 on 1 July 2001. Downloaded from SELF ASSESSMENT QUESTIONS A complicated case of von Hippel-Lindau disease G Thomas, R Hillson Answers on p 481. A 17 year old female shop assistant presented with a three week history of generalised headache, associated with nausea, vomiting, and vertigo. She had no past medical history, and was taking no regular medication. Her mother was currently receiving radiotherapy for anaplastic carcinoma of the thyroid gland. On examination she had an ataxic gait, bilateral papilloedema, and horizontal nystagmus to right lateral gaze. Left sided dysdiadokinesis and hyper-reflexia were demonstrated. Power and sensation were preserved. Computed tom- ography of the brain revealed a cystic lesion within the left cerebellum. Subsequent mag- netic resonance imaging (MRI) revealed a sec- ond, non-cystic lesion within the region of the right vermis (fig 1). Both images were consist- ent with cerebellar haemangioblastomata. A posterior fossa craniotomy was performed, with successful excision of both tumours. She made an uneventful recovery, with complete resolution of all symptoms, and was subse- Figure 3 MIBG isotope quently discharged. uptake scan. During a follow up outpatient appointment Figure 1 MRI of the brain. six months later, she complained of frequent panic attacks, associated with sweating and http://pmj.bmj.com/ palpitation that had begun two months earlier. These episodes were precipitated by exercise or excitement. She gave no history of heat intoler- Department of ance, weight loss, or diarrhoea. Examination Diabetes and was entirely normal. Abdominal computed Endocrinology, tomography (fig 2), and subsequent I131 meta- Hillingdon Hospital, iodobenzylguanidine (MIBG) isotope uptake Uxbridge, Middlesex, scan (fig 3) are shown here. on September 28, 2021 by guest. Protected copyright. UK G Thomas R Hillson Questions (1) What family history should be explored in Correspondence to: a 17 year old girl with multiple cerebellar Dr G Thomas, Flat 1, 134 haemangioblastomas and why? Shirland Road, Maida Vale, Figure 2 Computed tomography of the abdomen. London W9 2BT, UK (2) What is the nature of the current problem [email protected] and unifying diagnosis? (3) What other complications occur in this (4) What is the significance of thyroid cancer Submitted 2 September 1999 Accepted 4 April 2000 disease? in the mother? www.postgradmedj.com 472 Self assessment questions Answers on p 481. An unusual cause of paraplegia Postgrad Med J: first published as 10.1136/pmj.77.909.478 on 1 July 2001. Downloaded from University Department of Geriatric Medicine, North StaVs Combined S A M Saeed, D Francis, R Brooks Health Care NHS Trust, City General Hospital, Stoke-on-Trent, UK S A M Saeed A 56 year old machine operator presented with Neurological examination showed normal Queen Elizabeth mild weakness and sensory disturbance in his cranial nerves and normal upper limbs. Lower Medical Centre and lower limbs after he woke up from an arthros- limb examination revealed grade 3/4 proximal Walsall Manor copy operation. At the time he did not seek limb weakness with absent ankle jerks and Hospital, Walsall, West medical attention. His symptoms progressed equivocal plantar responses. Light touch and Midlands, UK D Francis over the next four months to the degree that he pinprick sensation was impaired in L1–S3 dis- R Brooks was unable to stand and he developed back tribution bilaterally with impaired vibration. pain radiating to the legs, urinary frequency, Coordination was normal. The remaining Correspondence to: DrSaadAMSaeed, hesitancy, and incontinence of urine. There physical examination was otherwise unremark- Springfield Unit, City was no history of claudication in the legs. He able. General Hospital, was a heavy smoker and suVered from Stoke-on-Trent, StaVs Laboratory examination showed high in- ST4 6QG, UK ischaemic heart disease and type 2 diabetes flammatory parameters with an erythrocyte [email protected] mellitus. sedimentation rate of 33 mm in the first hour Submitted 24 January 2000 On examination he was overweight. Straight and a C reactive protein of 68 mg/l. The Accepted 6 March 2000 leg raising test was within normal limits. following tests were either normal or negative: full blood count, biochemistry profile, immu- noglobulins, serum vitamin B12, serum and red blood cell folate, vasculitis, thrombophelia, and paraneoplastic screens, angiotensin con- verting enzyme level, and Treponema pallidum haemagglutination test. Lumbar puncture showed a cerebrospinal fluid (CSF) opening pressure of 260 mm with high CSF protein at 1.9 g/l (normal 0.1–0.4) with no evidence of oligoclonal band. CSF glu- cose 5.7 mmol/l (blood glucose 10.4 mmol/l), 73 red cells/high power field and no leucocytes or abnormal cells on cytospin. There was no evidence of xanthochromia. Computed tomography and magnetic reso- http://pmj.bmj.com/ nance imaging (MRI) scans of the brain were normal; however, an MRI scan of the spinal cord was abnormal (fig 1). After he was given specific medical treatment he became paraplegic (leg power 0/4) and lost bladder sensation. An urgent spinal angiogram confirmed the diagnosis (fig 2). Figure 1 (A) Axial and (B) sagittal MRI. T2 weighted scan of the spinal cord. He had specific interventional treatment on September 28, 2021 by guest. Protected copyright. after which he had some improvement in sensation in the lower limbs, only to be replaced by unpleasant dysthesia, but he started passing urine normally. Lower limb strength improved to grade 2–3/4, although he was not weight bearing. Eight months after the initial presentation he continued to improve, although he required a wheelchair for mobility. Questions (1) What diVerential clinical diagnoses would you consider? (2) What do the spinal MRI scans show? (3) What was the first treatment given which was followed by paraplegia? (4) What is the pathological abnormality sug- gested by the spinal angiogram? (5) What specific treatment did the patient receive? Figure 2 Spinal angiogram. Two pictures of the anterioposterior view. www.postgradmedj.com Self assessment questions 473 Palpable breast mass in a lactating woman Postgrad Med J: first published as 10.1136/pmj.77.909.478 on 1 July 2001. Downloaded from M Goyal, T B Poulton, O Konez Answers on p 482. A 35 year old woman noted a painless lump in her right breast during self examination. She was three months postpartum and had been breast feeding her daughter since birth. Her past medical history included three previous uneventful pregnancies, each followed by an uncomplicated period of breast feeding. She had no other significant illnesses in the past. Her mother had died of postmenopausal breast cancer. She had never been on oral contracep- tives. On examination, there was a mobile, non- tender, firm palpable mass of about 3 cm in the Figure 1 Longitudinal (left, with electronic cursors) and upper outer quadrant of the right breast, close transverse (right, with arrows) ultrasound images of the palpable breast mass showing a solid 2.5 cm long, ovoid to the nipple. There was no swelling or mass with its long axis parallel to the chest wall. Note the erythema of the overlying skin. Although the considerable posterior acoustic enhancement (arrowheads). Department of clinical impression favoured a benign breast sonographically normal. Transverse and longi- Radiology, Aultman mass, breast cancer was also considered. Hospital, 2600 Sixth tudinal ultrasound images of the mass are Street, SW, Canton, High resolution ultrasound of the right shown in fig 1. Ohio 44710, USA breast revealed a solid, predominantly hypoe- MGoyal choic mass with somewhat heterogeneous Questions T B Poulton echotexture. Some of the margins of the mass (1) What is the diVerential diagnosis of a O Konez were indistinct causing it to blend with the sur- solid mass in a pregnant or lactating Correspondence to: rounding breast parenchyma. The mass was woman? Dr Poulton ovoid, width greater than height, and there was (2) What further work-up is needed to con- [email protected] considerable posterior acoustic enhancement. firm the diagnosis in this case? Submitted 26 April 2000 There was no evidence of calcifications and the (3) What is the most likely diagnosis of this Accepted 9 May 2000 overlying skin and subcutaneous fat were patient’s palpable breast mass? http://pmj.bmj.com/ Answers on p 483. A patient with severe hyperphosphataemia Department of Medicine and KMChow,PKTLi Therapeutics, The on September 28, 2021 by guest. Protected copyright. Chinese University of Hong Kong, Prince of A 67 year old Chinese woman presented to her investigation revealed significant hyperphos- Wales Hospital, New general practitioner with abdominal pain. She phataemia, as shown in table 1. Further evalu- Territories, Hong Kong K M Chow had an unremarkable history except for bron- ation confirmed chronic renal failure second- PKTLi chiectasis diagnosed a decade before. Colonos- ary to polycystic kidney disease. copy was arranged in view of positive faecal Correspondence to: DrPhilipKTLi, occult blood. Preliminary investigation re- Department of Medicine and vealed underlying renal insuYciency with a Therapeutics, The Chinese serum creatinine concentration of 700 µmol/l University of Hong Kong, Prince of Wales Hospital, (table 1). There was no abnormality except Shatin, Hong Kong several small tubular adenomatous polyps in [email protected] the colon. Submitted 22 February 2000 She developed symptomatic hypocalcaemia Questions Accepted 3 April 2000 shortly after colonoscopy, and subsequent (1) What is the cause of the hyperphosphatae- mia? Table 1 Biochemical investigations (2) What action would you take? (3) How should this be avoided? One day after Reference Test Preliminary colonoscopy Six days later ranges Potassium (mmol/l) 3.06 2.8 3.4 3.5–5.1 Creatinine (µmol/l) 700 740 1130 80–140 Calcium (mmol/l) — 1.02 1.47 2.20–2.60 Phosphate (mmol/l) — 8.04 3.90 0.80–1.40 www.postgradmedj.com 474 Self assessment questions Abnormal behaviour in a man with massive, Postgrad Med J: first published as 10.1136/pmj.77.909.478 on 1 July 2001.
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