DOCSLIB.ORG

Neurosarcoidosis-Demonstration of Meningeal Disease by Gadolinium

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Neurosarcoidosis-Demonstration of Meningeal Disease by Gadolinium Journal ofNeurology, Neurosurgery, and Psychiatry 199 1;54:499-502 499 Neurosarcoidosis-demonstration of meningeal J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.54.6.499 on 1 June 1991. Downloaded from disease by gadolinium enhanced magnetic resonance imaging K T Khaw, H Manji, J Britton, F Schon Abstract (attributed to bilateral mastoidectomies) and Arriving at a firm diagnosis of neuro- left sided ninth to twelfth lower cranial nerve sarcoidosis continues to pose serious palsies. Cerebrospinal fluid examination was problems, particularly when evidence of unremarkable as was myelography and ver- granulomatous disease outside the tebral angiography. Biopsies taken of a nervous system is lacking. The common- possible mass in the left nasopharynx revealed est mode of presentation of neuro- no definite abnormality. Her erythrocyte sarcoidosis is with cranial nerve palsies. sedimentation rate was 40 mm/hr. Her Two cases of presumed neurosarcoidosis neurological condition again spontaneously with cranial nerve palsies showed clear improved. She remained well until the age of evidence of focal meningeal disease on 61 when a 5 x 7 cm mass was removed from gadolinium-DTPA enhanced MRI brain the left buttock which on histological examin- scans. Although not specific for sar- ation was consistent with erythema nodosum. coidosis, this technique may be very The patient then developed vaginal bleeding useful in aiding the diagnosis in sus- and other persistent gynaecological symp- pected cases. toms. These were treated surgically with hys- terectomy and bilateral salpingo-oophorec- Sarcoidosis is a disseminated disease of un- tomy at the age of 63 and colpectomy at the known aetiology characterised by non-caseat- age of 64. Histology from both these ing granulomatous infiltration. Neurological procedures revealed sterile non-caseating symptoms occur in about 5% of patients granulomatous tissue. The patient continued known to have the disease'2 and in up to 14% to have persistent vaginal ulceration and of patients at necropsy.' The commonest developed a urethro-vaginal fistula with mode of presentation of neurosarcoidosis is granulomatous pathology. with cranial nerve palsies.' Although facial At the age of 66 she had six episodes of nerve involvement, in particular, is frequently dysarthria, blurred vision with scintillating encountered, the pathophysiology of these teichopsia and numbness in her tongue lasting lesions is poorly understood, probably for about 30 minutes, without subsequent because they are associated with a good prog- headache or vomiting. Neurological examina- nosis. tion was unremarkable apart from severe http://jnnp.bmj.com/ Major diagnostic difficulties can arise, bilateral deafness and a diminished left-sided particularly in patients with no evidence of gag reflex. The diagnosis was felt to be granulomatous disease outside the nervous vertebrobasilar transient ischaemic attacks. system, because of problems in obtaining Investigations revealed an ESR of 46 with suitable tissue for histological examination. Diffuse meningeal infiltration particularly in at the skull base region is frequently found on September 27, 2021 by guest. Protected copyright. necropsy4 ' but is infrequently diagnosed positively during life, partly because until now imaging techniques have usually not shown up such meningeal involvement. This paper describes two patients with presumed neurosarcoidosis which with cranial Atkinson Morley's presented Hospital, London, UK nerve palsies, in whom evidence of focal Department of meningeal disease was demonstrated on Neuroradiology gadolinium-DTPA (Gd-DTPA) enhanced K T Khaw J Britton MRI. Department of Neurology Case 1 H Manji A 65 year old white woman presented at the F Schon age of 39 with right sided pleuritic chest pain Correspondence to: Dr Schon, Department of and an abnormal chest radiograph showing an Neurology, Atkinson irregular mass in the right apex. Despite Morley's Hospital, Copse Hill, Wimbledon, London extensive investigations no firm diagnosis was SW20 ONE, UK made and her condition spontaneously Received 3 April 1990 improved. At the age of 43 she developed and in revised form Figure 1 Case 1. Enhanced axial CT scan through 12 October 1990. headaches, dysphagia and dysphonia. She was posteriorfossa showing enhancement of the tentorium Accepted 7 November 1990 found to have bilateral conductive deafness cerebelli on the left. 500 Khaw, Manji, Britton, Schon elevated serum calcium of 2 79 mmol/l. A Case 2 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.54.6.499 on 1 June 1991. Downloaded from chest radiograph showed a small calcified A 53 year old businessman who had been born lesion in the left lower zone only. Serum in India but had lived in England for twenty angiotensin converting enzyme was normal. years developed double vision in 1985, which CT scan showed mild asymmetrical dilatation on examination was due to a partial pupil of the lateral ventricles and a possible mass in sparing right sided third nerve palsy. Extensive the left posterior fossa with contrast investigations were all unremarkable including enhancement of the tentorium cerebelli on the erythrocyte sedimentation rate, chest left (fig 1). An MRI scan was obtained using a radiograph and enhanced CT brain scan. Siemens Magnetom 15 Tesla scanner. A T2 Although no definite diagnosis was made at weighted sequence (TR 3-2 s TE 90 ms) that time, the patient was treated with a short showed no evidence of a mass lesion (fig 2a) course of oral prednisolone (40 mg for two but confirmed mild asymmetrical hydro- weeks) and all his symptoms and signs fully cephalus and demonstrated a Chiari malfor- resolved. mation. In addition abnormal bright He remained well till June 1989 when he enhancement of the meninges of the left again complained of double vision, which on cerebral hemisphere, tentorium cerebelli and this occasion was due to a complete right-sided left side of the posterior fossa was shown on lateral rectus palsy. Whilst he was being inves- Gd-DTPA enhanced Ti weighted sequences tigated for this isolated cranial nerve palsy, he (TR 0 5 s TE 17 ms) carried out in the gradually developed right facial numbness, coronal and axial planes (fig 2b, c). Unenhan- increasing hoarseness of his voice and ced Ti weighted images were not obtained as dysphagia over a six week period. Examination a bright sequence from the meninges would revealed multiple right-sided cranial nerve indicate a fairly acute bleed which was clin- palsies involving the fifth, sixth, ninth and tenth ically highly unlikely. nerves. At the age of 67 she developed epilepsy with Investigations revealed an ESR of 55 mm/hr eight attacks of right sided jerking followed by but normal serum calcium and angiotensin loss of consciousness for up to 20 minutes. converting enzyme levels. Repeat chest These attacks stopped after treatment with radiograph and CT brain scans were again phenytoin was started. The EEG was un- normal. Cerebrospinal fluid examination remarkable. The Mantoux test was negative at showed only mild abnormalities-six 1:10,000 dilution, Kveim test was negative. lymphocytes, protein 0-84 g/l and glucose 4 1 No repeat CSF examination was carried out. mmol/l. Acid-fast bacilli were not seen or This woman had histologically proven non- cultured from the spinal fluid and cytological caseating granulomatous disease of the female examination confirmed the absence of malig- genital tract and of a subcutaneous buttock nant cells. The Mantoux test was clearly mass. Sarcoidosis affecting the uterus is well positive at a dilution of 1:10,000. A Kveim test recognised but in fact few proven cases have was not carried out. Gallium scanning showed been reported.' In addition she had evidence marked bilateral lacrimal gland and bilateral of neurological involvement, having had an hilar lymph node uptake, a pattern well recog- episode of cranial nerve palsies and epilepsy, nised in sarcoidosis.7 MRI scan with Gd- the latter being reported in up to 35% of cases DTPA enhancement showed clearcut abnor- http://jnnp.bmj.com/ of granulomatous meningitis.6 mal enhancement affecting only the meninges on September 27, 2021 by guest. Protected copyright. Figure 2 Case 1. a) T2 weighted (TR 3-2 s TE 90 ms) axial magnetic resonance images obtained at the level of the pons showing no mass lesion or area of abnormal signal. The CSF returns a normal high signal on this sequence. b) axial and c) coronal Gadolinium-DTPA enhanced Ti (TR 0 5 s TE 17 ms) weighted MRI scans showing bright meningeal enhancement over 1'ft hemisphere and tentorium cerebelli. Neurosarcoidosis-demonstration of meningeal disease by gadolinium enhanced magnetic resonance imaging 501 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.54.6.499 on 1 June 1991. Downloaded from Figure 3 Case 2. a) Axial and b) and c) coronal Gd-DTPA enhanced Tl weighted MRI scans showing enhancement of the tentorium cerebelli on the right. d) and e) Axial T2 weighted MRI scans again showing no abnormality in the brain stem or CSF. on the right side maximal in the region of the scan, the lymphocytic CSF and the lack of any tentorium cerebelli (fig 3a, b, c). T2 weighted evidence over many years of follow up of other sequences using the same sequences as before major causes of meningeal disease such as showed no abnormality in the brain stem or carcinoma and tuberculosis. http://jnnp.bmj.com/ adjacent CSF (figs 3d, e). In sarcoidosis of the central nervous system, This patient presented with two episodes of leptomeningeal disease is the commonest cranial nerve palsies without any clinical pattern of involvement seen, and may be local- evidence of systemic disease outside the central ised or widespread, resulting in focal or dis- nervous system. The illness was felt to be due seminated meningeal nodules or plaques.2 This to sarcoidosis although tuberculosis was may result in basal obstruction of ventricular obviously an important differential diagnosis.
Load more

Recommended publications
  • Consequences of Sarcoidosis
    Consequences of Sarcoidosis Marjolein Drent, MD, PhDa,b,c,*, Bert Strookappe, MScc,d, Elske Hoitsma, MD, PhDc,e, Jolanda De Vries, MSc, PhDc,f,g KEYWORDS Cognitive impairment Depressive symptoms Exercise limitation Fatigue Pain Rehabilitation Sarcoidosis Small fiber neuropathy Quality of life KEY POINTS Consequences of sarcoidosis are wide ranging, and have a great impact on patients’ lives. Sarcoidosis patients suffer not only from organ-related symptoms, but also from a wide spectrum of rather nonspecific disabling symptoms. Absence of evidence does not mean evidence of absence. Management of sarcoidosis requires a multidisciplinary personalized approach that focuses on somatic as well as psychosocial aspects of the disease. INTRODUCTION of sarcoidosis patients include symptoms that cannot be explained by granulomatous involve- The clinical expression, natural history, and prog- ment of a particular organ.4 Apart from lung- nosis of sarcoidosis are highly variable and its related symptoms (eg, coughing, breathlessness, course is often unpredictable.1 Clinical manifesta- 1,2 and dyspnea on exertion), patients may suffer tions vary with the organs involved. The lungs from a wide range of rather nonspecific disabling are affected in approximately 90% of patients symptoms.2,5 These symptoms, such as fatigue, with sarcoidosis, and the disease frequently also fever, anorexia, arthralgia, muscle pain, general involves the lymph nodes, skin, and eyes. Remis- weakness, muscle weakness, exercise limitation, sion occurs in more than one-half of patients within and cognitive failure, often do not correspond 3 years of diagnosis, and within 10 years in two- 2,5–9 2 with objective physical evidence of disease. thirds, with few or no remaining consequences.
    [Show full text]
  • An Elderly Patient with Sarcoidosis Manifesting Panhypopituitarism with Central Diabetes Insipidus
    Endocrine Journal 2007, 54 (3), 425–430 An Elderly Patient with Sarcoidosis Manifesting Panhypopituitarism with Central Diabetes Insipidus TOMOKO MIYOSHI, FUMIO OTSUKA, MASAYA TAKEDA, KENICHI INAGAKI, HIROYUKI OTANI, TOSHIO OGURA, KEN ICHIKI*, TETSUKI AMANO* AND HIROFUMI MAKINO Department of Medicine and Clinical Science, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, 2-5-1 Shikata-cho, Okayama City, 700-8558, Japan *Aioi City Hospital, 5-12 Sakae-cho, Aioi City, 678-0008, Japan Abstract. We here report a 77-year-old Japanese male who suffered general fatigue with progressive thirst and polyuria. Central diabetes insipidus was diagnosed by depletion of vasopressin secretion in response to increases in serum osmolality. Secretory responses of anterior pituitary hormones including adrenocorticotropin, thyrotropin, gonadotropins and growth hormone were severely impaired. Diffuse swelling of the infundibulum as well as lack of T1-hyperintense signal in the posterior lobe was noted by pituitary magnetic resonance imaging. The presence of bilateral hilar lymphade- nopathy and increased CD4/CD8 ratio in bronchoalveolar lavage fluid was diagnostic for lung sarcoidosis. Physiological doses of corticosteroid and thyroid hormone were administered in addition to desmopressin supplementation. Complete regression of the neurohypophysial swelling was notable two years after corticosteroid replacement. Diffuse damage of anterior pituitary combined with hypothalamic involvement leading to central diabetes insipidus is a rare manifestation in such elderly patients with neurosarcoidosis. Key words: Central diabetes insipidus, Hypophysitis, Lymphocytic infundibuloneurohypophysitis, Neurosarcoidosis, Panhypopituitarism, Sarcoidosis (Endocrine Journal 54: 425–430, 2007) SARCOIDOSIS is a systemic granulomatous disease dysfunction and less frequently involve the infundibu- involving multiple organs, in which endocrinopathy is lum and/or the pituitary gland, leading to hypothalamic rarely complicated [1].
    [Show full text]
  • Coexistence of Neurosarcoidosis and Multiple Sclerosis
    Neurol. Croat. Vol. 61, 3-4, 2012 Coexistence of neurosarcoidosis and multiple sclerosis L. Radolović Prenc1, S.Telarović2,3, I.Vidović1, J. Sepčić4, L. Labinac Peteh1 ABSTRACT - Sarcoidosis is a multisystem infl ammatory disease of unknown etiology that predominantly aff ects the lungs and intrathoracic lymph nodes, but in 6% of cases it also occurs in central or peripheral nervous system. Multiple sclerosis (MS) is an immune-mediated infl ammatory disease that attacks myeli- nated axons in the central nervous system. Coexistence of sarcoidosis and other autoimmune diseases like MS is rarely reported in the literature. We present a case report of a patient with coexisting sarcoidosis and 67 MS, with a positive family history of MS. Symptoms of sarcoidosis appeared three years before the onset of Number 3-4, 2012 Number symptoms typical for MS. Similarity of demyelinating lesions in the nervous system, increased IgG in cere- brospinal fl uid and good response to corticosteroid treatment point to similar etiology. Th e onset of diseases like sarcoidosis and MS in the same patient over a period of only a few years opens the question whether the two separate entities come in sequence or the onset of sarcoidosis occurs during development of typical clinical presentation of MS. Key words: sarcoidosis, encephalomyelitis, multiple sclerosis INTRODUCTION lymph nodes (Figs. 1 and 2), or pathologic skin nodes or bone cysts, especially in hands, and posi- Sarcoidosis is an infl ammatory disorder of un- tive Kveim test (2). Cerebrospinal fl uid (CSF) anal- known origin, characterized by epithelioid cell ysis shows increased IgG, pleocytosis with protein- granulomas in various organs (1).
    [Show full text]
  • Neurosarcoidosis
    CHAPTER 11 Neurosarcoidosis E. Hoitsma*,#, O.P. Sharma} *Dept of Neurology and #Sarcoidosis Management Centre, University Hospital Maastricht, Maastricht, The Netherlands, and }Dept of Pulmonary and Critical Care Medicine, Keck School of Medicine, University of Southern California, Los Angeles, CA, USA. Correspondence: O.P. Sharma, Room 11-900, LACzUSC Medical Center, 1200 North State Street, Los Angeles, CA 90033, USA. Fax: 1 3232262728; E-mail: [email protected] Sarcoidosis is an inflammatory multisystemic disorder. Its cause is not known. The disease may involve any part of the nervous system. The incidence of clinical involvement of the nervous system in a sarcoidosis population is estimated to be y5–15% [1, 2]. However, the incidence of subclinical neurosarcoidosis may be much higher [3, 4]. Necropsy studies suggest that ante mortem diagnosis is made in only 50% of patients with nervous system involvement [5]. As neurosarcoidosis may manifest itself in many different ways, diagnosis may be complicated [2, 3, 6–10]. It may appear in an acute explosive fashion or as a slow chronic illness. Furthermore, any part of the nervous system can be attacked by sarcoidosis, but the cranial nerves, hypothalamus and pituitary gland are more commonly involved [1]. Sarcoid granulomas can affect the meninges, parenchyma of the brain, hypothalamus, brainstem, subependymal layer of the ventricular system, choroid plexuses and peripheral nerves, and also the blood vessels supplying the nervous structures [11, 12]. One-third of neurosarcoidosis patients show multiple neurological lesions. If neurological syndromes develop in a patient with biopsy- proven active systemic sarcoidosis, the diagnosis is usually easy. However, without biopsy evidence of sarcoidosis at other sites, nervous system sarcoidosis remains a difficult diagnosis [13].
    [Show full text]
  • The Pathogenesis and Treatment of Optic Disc Swelling in Neurosarcoidosis a Unique Therapeutic Response to Infliximab
    OBSERVATION The Pathogenesis and Treatment of Optic Disc Swelling in Neurosarcoidosis A Unique Therapeutic Response to Infliximab Jeffrey M. Katz, MD; Michiko Kimura Bruno, MD; Jacqueline M. S. Winterkorn, MD, PhD; Nancy Nealon, MD Objective: To review the pathogenesis and treatment eye. A 57-year-old woman presented with bilateral, sub- of optic disc swelling in neurosarcoidosis, including a acute, painful visual loss and unilateral papillitis consis- novel therapeutic response to infliximab. tent with optic neuritis. Her visual loss responded rap- idly to intravenous corticosteroids. The funduscopic Design and Setting: Case reports from an inpatient examination findings in both patients prompted further neurology service. clinical investigation, culminating in the diagnosis of neu- rosarcoidosis. Patients: A 35-year-old woman presented with head- ache, chronic visual loss, papilledema, and optic atro- Conclusion: Understanding the multiple etiologic mecha- phy, characteristic of chronic intracranial hypertension. nisms that produce optic disc swelling in sarcoidosis can Magnetic resonance imaging showed bifrontal cerebral help neurologists tailor treatment for patients with neu- edema with en plaque frontal pachymeningeal enhance- rosarcoidosis who present with this symptom. ment. Her visual loss progressed despite conventional therapies. The use of the tumor necrosis factor ␣ antago- nist infliximab maintained functional vision in her right Arch Neurol. 2003;60:426-430 OSS OF VISION associated with 20/25 OD with a constricted visual field (VF) optic disc swelling (ODS) is and an inferonasal step. The left eye had no a rare initial presentation of light perception and an amaurotic pupil. neurosarcoidosis. Optic disc Funduscopic examination findings re- swelling is an important vealed right optic disc swelling (Figure 1A) Lclinical sign because it can herald central and left optic disc pallor and resolving swell- nervous system disease in an otherwise ing (Figure 1B).
    [Show full text]
  • Sarcoidosis Optic Neuropathy
    Sarcoidosis Optic Neuropathy Optometric Retina Society Residency Award Delia Groshek, OD White River Junction VA Medical Center Ocular Disease Residency 2010-2011 6244 North Knox Avenue Chicago, IL 60646 (773) 620-5993 [email protected] Abstract Sarcoidosis is a multi-systemic granulomatous disease of unknown etiology that can affect almost every organ in the body, though it typically affects the lung, lymph nodes, skin, liver and eyes. The diagnosis of sarcoidosis is based on histological evidence of noncaseating epithelioid cell granulomas, bilateral hilar lymphadenopathy, and exclusion of other diseases that produce a similar clinical and/or histological picture. Ocular involvement may be the presenting sign of the disease and can involve any ocular structure. In this case, a 67 year old Caucasian male presents with blur, photopsia, papillitis, and periphlebitis. This case report reviews the diagnosis, treatment and management of ocular sarcoidosis. Key words: angiotension converting enzyme, bilateral hilar lymphadenopathy, noncaseating granuloma, papillitis, periphlebitis, sarcoidosis Case Report A 67 year old Caucasian male presented to the VA eye clinic with a chief complaint of central blurring of his vision in his left eye, which happened twice the previous night lasting for five minutes each time. Afterwards, he saw blue spots. In the exam, he stated his left eye felt like it had a dull, aching pain of a 2-3 on a scale of 10. He reported itching and tearing of his left eye. Upon further questioning, the patient denied scalp tenderness, jaw claudication, or recent weight loss. The patient did report neck pain, which has been stable for years due to his arthritis.
    [Show full text]
  • Neurosarcoidosis: Clinical Manifestations, Investigation and Treatment
    REVIEW Pract Neurol: first published as 10.1136/practneurol-2019-002349 on 17 May 2020. Downloaded from Neurosarcoidosis: clinical manifestations, investigation and treatment Desmond P Kidd Centre for Neurosarcoidosis, ABSTRACT eye may be involved, and skin, cardiac, Neuroimmunology unit, Sarcoidosis affects the nervous system in 10% hepatic, renal, bone and joint involve- Institute of Immunology and 3 Transplantation, University of cases. When it does so it can affect any part ment is common. College London, London, UK of the nervous system and with all degrees Around 30% of cases resolve within of severity. It forms part of the differential 2 years, particularly with single system Correspondence to Dr Desmond P Kidd, Royal Free diagnosis in inflammatory, infective, neoplastic involvement, 30% have a relapsing form Hospital, London NW3 2QG, UK; and degenerative neurological diseases and and 30% progressively deteriorate. d. kidd@ ucl. ac. uk may be very difficult to diagnose without histological confirmation. Recent clinical studies Accepted 5 January 2020 Epidemiology and the increasing availability of new biological Differences in the prevalence of the treatments allow a much clearer understanding disease have been known for decades; it of the disease. This review summarises its clinical has long been known that the incidence features, imaging and laboratory characteristics, is highest in Nordic countries 11–24 treatment and outcome. per 105,4 and lowest in south- east Asian countries (0.85 in Korea, 2.17 in Taiwan and 1.01 in Japan).5–8 African Americans INTRODUCTION within the USA have three times the inci- Sarcoidosis is an auto-inflammatory dence of European Americans,9 and the disorder characterised by the develop- incidence is lower still in Hispanic Amer- ment of granulomatous inflammation in icans.10 Recent studies from the Swedish affected tissues.
    [Show full text]
  • Neurosarcoidosis Manifesting As Tremor of the Extremities and Severe Hypopituitarism —Case Report—
    Neurol Med Chir (Tokyo) 48, 314¿317, 2008 Neurosarcoidosis Manifesting as Tremor of the Extremities and Severe Hypopituitarism —Case Report— Yoshikazu OGAWA,TeijiTOMINAGA*,andHidetoshiIKEDA** Department of Neurosurgery, Kohnan Hospital, Sendai, Miyagi; *Department of Neurosurgery, Tohoku Graduate School of Medicine, Sendai, Miyagi; **Department of Neurosurgery, Ohara Medical Center, Fukushima Abstract A 48-year-old woman initially presented with significant tremor of the extremities and subsequent severe hypopituitarism. Magnetic resonance imaging showed hyperintense areas in bilateral caudate heads and putamina, and a pituitary mass. L-dopa and corticosteroid were given and the tremor was reduced. Serum markers including autoimmune diseases were negative. Computed tomography and positron emission tomography detected no abnormalities except for pituitary lesion. Transsphenoidal biopsy revealed a noncaseating granuloma including giant cells with destroyed pituitary gland. The diagnosis was sarcoidosis. Diagnosis of isolated neurosarcoidosis is definitely difficult. Biopsy may be essential to establish the diagnosis in such a case. Corticosteroid administration is strongly recom- mended to avoid irreversible damage to the normal tissues even if histological confirmation was not achieved. Key words: basal ganglia, hypopituitarism, neurosarcoidosis, tremor Introduction Case Presentation Neurosarcoidosis accounts for about 5% of cases of A 48-year-old woman suffering from severe tremor systemic sarcoidosis.2) The major types of clinical of
    [Show full text]
  • Sarcoidosis Presenting As Longitudinally Extensive
    RESIDENT & FELLOW SECTION Teaching NeuroImages: Section Editor Sarcoidosis presenting as longitudinally Mitchell S.V. Elkind, MD, MS extensive myelitis Excellent response to infliximab Naila Goenka, MD Nagagopal Venna, MD Figure 1 Pretreatment MRI Figure 2 Posttreatment MRI Correspondence to Dr. Goenka: [email protected] Sagittal T2-weighted image (A) demonstrates confluent hy- Sagittal T2-weighted image (A) demonstrates complete res- perintensity extending from the upper cervical to the upper olution of the hyperintensity and associated resolution of thoracic spinal cord. On postgadolinium T1-weighted image the postgadolinium enhancement (B). (B), there is multifocal, patchy enhancement. A 44-year-old man presented with 2 weeks of pro- AUTHOR CONTRIBUTIONS gressive right body numbness, weakness, and cir- N.G. reviewed the clinical case and prepared the manuscript and figures. N.V. supervised the clinical care and commented critically on the man- cumferential burning sensation around his trunk. uscript. Both authors approved the final version. MRI spine demonstrated longitudinally extensive myelitis (figure 1). MRI brain and laboratory in- STUDY FUNDING vestigations were normal except for CSF lympho- No targeted funding reported. cytic pleocytosis (9 leukocytes/mm3). CT revealed DISCLOSURE hilar and mediastinal lymphadenopathy, which on The authors report no disclosures relevant to the manuscript. Go to biopsy showed noncaseating granulomas sugges- Neurology.org for full disclosures. tive of sarcoidosis. As the patient had multiple clinical relapses on steroids, he was treated with REFERENCES infliximab infusions (5 mg/kg every 4 weeks) and 1. Dolhun R, Sriram S. Neurosarcoidosis presenting as longitu- dinally extensive transverse myelitis. J Clin Neurosci 2009;16: had complete clinical and radiologic resolution 595–597.
    [Show full text]
  • Aseptic Meningitis and Hydrocephalus Secondary to Neurosarcoidosis Anmol Pandey,1 Thomas Stoker,1 Lukasz a Adamczyk,2 Sybil Stacpoole3
    Case report BMJ Case Rep: first published as 10.1136/bcr-2021-242312 on 26 August 2021. Downloaded from Aseptic meningitis and hydrocephalus secondary to neurosarcoidosis Anmol Pandey,1 Thomas Stoker,1 Lukasz A Adamczyk,2 Sybil Stacpoole3 1Department of Neurology, The SUMMARY normal aside from a mild lymphopaenia which was National Hospital for Neurology A 53- year- old woman presented to hospital with gait present from the onset of her symptoms in May. and Neurosurgery, UCL Queen instability, urinary incontinence and confusion. She had a Serum Angiotensin Converting Enzyme (ACE) was Square Institute of Neurology, 4- month history of headache, blurred vision, personality non- elevated at <12 units/L (normal range (NR): University College London change and memory problems. Magnetic Resonance 20–70). Her Addenbrooke’s Cognitive Examina- Hospitals NHS Foundation Trust, tion–Revised (ACE-R) score was 45/100. Her cere- London, UK Imaging of the brain after contrast application 2Department of Histopathology, showed tectal plate and occipital enhancement, brospinal fluid (CSF) showed an elevated white cell Peterborough City Hospital, as well as a known hydrocephalus. Cerebrospinal count of 60 cells/µL (NR: 0–5), an elevated protein North West Anglia NHS fluid showed aseptic meningitis with no evidence of of 1.04 g/L (normal range: 0.15–0.45) and a low Foundation Trust, Peterborough, clonal expansion. After further imaging that showed glucose of 1.6 mmol/L (NR: 2.2–4.0). Subsequent UK generalised lymphadenopathy and subsequent tissue CSF flow cytometry confirmed lymphocytosis with 3Department of Neurology, biopsy that showed granulomatous lymphadenitis, she T- cell predominance with no phenotypic evidence Peterborough City Hospital, was diagnosed with neurosarcoidosis.
    [Show full text]
  • Sarcoidosis Treatment Guidelines
    SARCOIDOSIS TREATMENT GUIDELINES INTRODUCTION Goals of Sarcoidosis Management Sarcoidosis is a chronic inflammatory granulomatous dis- The goals of sarcoidosis management are to prevent or con- ease that primarily affects the lungs, although multi-organ trol organ damage, relieve symptoms and improve the involvement is common. The etiology of sarcoidosis is not patient’s quality of life. An evaluation by a pulmonologist is clear; however, genetic and environmental factors probably strongly recommended. For patients with extrapulmonary play a role in the development and expression of the disease. involvement, a multidisciplinary approach may be required. A patient may need to see an ophthalmologist for ocular dis- Once thought to be rare, sarcoidosis affects people ease, a cardiologist for cardiac disease, a neurologist for neu- throughout the world. It can affect people of any age, race, rological disease, a nephrologist for renal disease, and so or gender; however, the prevalence is highest among adults forth. between the ages of 20 and 40 and in African Americans and people of European – particularly Scandinavian – descent. Pharmacologic Treatment Symptoms and severity can vary by race and gender, with While a significant percentage of sarcoidosis patients never African Americans being more severely affected than need therapy, there are several groups which require treat- Caucasians. Extrapulmonary sarcoidosis is common in cer- ment. In this monograph, we will discuss several of the com- tain populations, for example: chronic uveitis in African monly used drugs for sarcoidosis and their potential toxici- Americans, painful skin lesions in Northern Europeans and ties, and will provide algorithms for use of these drugs to cardiac and ocular involvement in Japanese.
    [Show full text]
  • PAPILLOEDEMA in SARCOIDOSIS*T by D
    Br J Ophthalmol: first published as 10.1136/bjo.51.8.526 on 1 August 1967. Downloaded from Brit. J. Ophthal. (1967) 51, 526 PAPILLOEDEMA IN SARCOIDOSIS*t BY D. GERAINT JAMES, M. A. ZATOUROFF, JOAN TROWELL From the Royal Northern Hospital, London. AND F. CLIFFORD ROSE Charing Cross Hospital, London POSTERIOR uveitis is not a frequent presentation of ocular sarcoidosis because it is usually obscured by overlying anterior uveitis (James, Anderson, Langley, and Ainslie, 1964), so that involvement of the optic disc is rarely recognized as a feature of sarcoidosis. Now that local and systemic corticosteroids are quickly resolving acute inflammation of the anterior segment of the eye, ophthalmoscopic examination is revealing hitherto unsuspected lesions of the posterior segment. In a personally studied series of 422 patients with histologically confirmed sarcoidosis, 112 (26 per cent.) had ocular and 31 (7 per cent.) neurological involvement. Amongst these patients were four with papilloedema (Table); two were intensively investigated at the onset for evidence of a cerebral space-occupying lesion rather than for neurological sarcoidosis. The third case illustrates that awareness of neurosarcoidosis can avoid needless and unfruitful neurological investigation. TABLE FEATURES OF 4 PATIENTS WITH PAPILLOEDEMA DUE TO SARCOIDOSIS Age Cerebrospinal Fluid Clinical Accompaniments Case at t Chest Kveim Mantoux Other http://bjo.bmj.com/ No. Sex Onset Pressure Protein Cells Facial Iritis Other r Test Test Histology yr) (mm.) (mg.) ClsPalsy Iii te a Paraesthesiae oftrunk 1 F 27 130 70 8 + + Loss oftaste, smell + - Skin left High tone deafness Rash 2 M 49 90 60 10 + No 6th nerve palsy Mott- + - Liver left Hypercalcaemia ling Lymph node on October 3, 2021 by guest.
    [Show full text]