DOCSLIB.ORG

1050 Neurosarcoidosis Presenting As Major Depression Letters

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text

Load more

J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.47.9.1050 on 1 September 1984. Downloaded from 1050 Letters embedding it remains feasible that the two of three objects after five minutes. or the major sign of an exacerbation.36 virus that caused encephalitis lethargica (if There were no clear-cut cognitive deficits Gilmore et a13 reported a patient with virus it was) may yet be identified in mater- noted. known sarcoidosis thought to be in remis- ial that has been stored since the time of Outpatient treatment with imipramine sion who had a depressive episode without the epidemic. was begun for major depression. This other evidence of active sarcoidosis. The therapy was discontinued because of into- cerebrospinal fluid was suggestive of active MM ESIRI lerable lethargy. The patient was then disease. In Hook's4 report of nine cases of The Radcliffe Infirmary, admitted to the inpatient psychiatric unit neurosarcoidosis, case number 2 was diag- Oxford OX26 6HE for electroconvulsive therapy. At that time nosed as depression. Zerman6 described a her mental status examination was mixed psychiatric-neurologic syndrome M SWASH unchanged and the remainder of her with features of Wernicke-Korsakoff London Hospital, neurological exam was reported as normal. psychosis as the initial manifestation of sar- Whitechapel, After eight treatments (one bilateral and coidosis. Suchenwirth and Dold5 described London UK seven unilateral) she became severely con- a man with a paranoid psychosis who was fused with urinary incontinence and found to have sarcoidosis. His psychiatric difficulty in walking. Neurological consul- symptoms responded to steroid medica- tation was requested. On examination, she tion. References was disoriented to time, place, and could Our patient initially had no clear evi- not identify her psychiatrist. There were no dence of active sarcoidosis. The clinical pic- 'Flexner S. Epidemic (lethargic) encephalitis motor or sensory abnormalities other than ture of and allied conditions. JAMA 1923; tearfulness, constricted affect, 81:1688-93; 1785-9. a slightly wide-based, unsteady gait. An anhedonia, and disturbance of sleep and 2 Levaditi C, Harvier P, Nicolam P. Etude erythematous malar rash was noted. Inves- appetite supported a diagnosis of a major exp6rimentale de l'enc6phalitie dite 'ldthar- tigation of the cause of her encephalopathy depressive episode. Following ECT, the gique'. Ann Inst Past 1922;36:63-148. showed cerebrospinal fluid (CSF) to con- profound organic mental syndrome, gait 3Esiri MM. Herpes simplex encephalitis: an tain protein 160 mg/dl, glucose 40 mg/dl, disorder, and urinary incontinence indi- immunohistological study of the distribution 22 WBC/mm3 with 83% lymphocytes, cated the organic basis of the disease and of viral antigen within the brain. J Neurol 15% monocytes, 2% polymorphonuclear prompted the neurologic consultation. TheProtected by copyright. Sci 1982;54:209-26. cells, negative VDRL, negative bacterial diagnosis of was on 4 Kennard C, Swash M. Acute viral encephalitis: neurosarcoidosis based its diagnosis and outcome. Brain and fungal cultures, and no malignant cells. the positive skin biopsy, positive Kveim 1981; 104:129-48. An electroencephalogram showed test and cerebrospinal fluid formula of ele- increased diffuse activity in the theta and vated protein, low glucose, and a mild, delta frequencies. The CT scan showed predominantly lymphocytic pleocytosis. As Neurosarcoidosis presenting as major decreased attenuation in the white matter with Suchenwirth and Dold's5 case and depression adjacent to the lateral ventricles most other patients with dementia due to sar- prominently in the frontal region, and a coid,2 our patient had a favorable clinical Sir: Neurological complications of sar- cerebral angiogram was normal with no response to steroid medication with coidosis occur in approximately 5% of all evidence of vasculopathy. Blood chemistry significant improvement of the CSF cases.' Rarely, dementia mimicking CBC, ANA, ESR, and thyroid function abnormalities. Alzheimer's disease may be the presenting studies were all normal. The CSF findings The CT scan showed decreased attenua- or prominent manifestation of the illness.2 from a second lumbar puncture were: pro- tion of the periventricular white matter, Less commonly, prominent psychiatric tein 139 mg/dl, glucose 46 mg/dl, 21 WBC/ primarily in the frontal region. The rele- manifestations in relative isolation can mm3 with 83% lymphocytes, 14% mono- vance of this is not clear. CT findings in occur at the onset or during an exacerba- cytes, 3% polymorphonuclear cells, and no intracranial neurosarcoidosis are non- tion of the disease.23 We describe a patient malignant cells. A skin biopsy taken in the specific. They include normal scans, hyd- who ultimately proved to have neurosar- area of the malar rash showed non- rocephalus, abnormal enhancement of the coidosis and whose clinical presentation caseating granulomata. Kveim-Siltzbach meninges (especially in the basal area), dif- was indistinguishable from a major depres- antigen was injected and the biopsy several fuse enhancement throughout the entirehttp://jnnp.bmj.com/ sive episode. weeks later was positive for sarcoidosis. cerebrum and intraparenchymal mass A 57-year-old chronically dysphoric There was no definitive evidence of sarco- lesions which often have a somewhat woman complained to her psychiatrist of idosis on several chest radiographs. increased density compared to brain tissue deteriorating memory and concentration The patient was treated with corticos- and show enhancement on contrast which forced her to give up her job as an teroids (prednisone 60 mg/day). Within studies.''3 The association of sarcoidosis office manager. Initial examination by her three weeks of starting treatment her think- and progressive multifocal leukoence- psychiatrist showed her to be oriented and ing was more lucid and her mood brighter. phalopathy is known.' In our patient this coherent but tearful, with depressed mood CSF findings at that time were: protein 68 possibility cannot be entirely ruled out. and constricted affect. Her thought content mg/dl, glucose 70 mg/dl, 9 WBC/mm3 with However, it is unlikely because of the on October 1, 2021 by guest. included feelings of helplessness, hopeless- 81% lymphocytes, 9% monocytes, 10% improvement in clinical status and CSF ness, anhedonia, and complaints of insom- polymorphonuclear cells. A repeat CT scan abnormalities with steroids. In addition, nia and anorexia causing a 10 pound (4.5 was unchanged. the usual CT findings of low density lesions kg) weight loss. She named the past four There are four reported cases in the lit- of the central and convolutional white mat- presidents of the USA, spelled a four letter erature of sarcoidosis in which a psychiatric ter with scalloped lateral borders were not word forwards and backwards, and recalled syndrome was the presenting manifestation present.'4 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.47.9.1050 on 1 September 1984. Downloaded from Letters 1051 These cases illustrate that the principal inflammation and focal hydrocephalus clinical manifestations of sarcoidosis may shown by sequential CT. Neurol (Minneap) 1979;29: 1161-65. on rare occasions be psychiatric and 12 Kendall BE, Tatler GLV. Radiological findings emphasise the importance of the possibility in neurosarcoidosis. Br J Radiology of effective treatment with steroids. 1978;51:81-92. 3 Brooke SB, El Hammal T, Hungerford GD, We are grateful to Dr Stefan Shanzer for Acker J, Trevor RP, Russel W. Radiologic review of this manuscript. evaluation of neurosarcoidosis: role of computed tomography. Am J Neuroradiol- JOHN STILLER* ogy 1982;3:513-21. Caroll BA, Barton L, Norman D, Engmann D. ANDREW GOODMAN* Diagnosis of progressive multifocal LAWRENCE M KAMHIt leukoencephalopathy by computed tomog- MICHAEL SACHER* raphy. Radiology 1977; 122:137-41. MORRIS B BENDERO Accepted 17 March 1984 The Departments ofNeurology, * Psychiatry, t and Radiology, t The Mount Sinai Medical Center, New York, and The Mount Sinai School ofMedicine ofthe City University ofNew York. Address for reprint requests: Dr John Stil- ler, Department of Neurology, 2150 Penn- sylvania Avenue, NW Washington, DC 20037, USA §deceased Protected by copyright. References Delaney P. Neurologic manifestations in sar- coidosis: review of the literature with a report of 23 cases. Ann Intern Med 1977;87:336-45. 2 Cordingly G, Navano C, Brust JCM, Healton ET. Sarcoidosis presenting as dementia. Neurol (NY) 198 1; 31: 1148-51. 3Gilmore K, Rudden M, Kalman TP. Psychiatric manifestations of sarcoidosis. Can J Psychiatry 1980; 25:329-31. Hook 0. Sarcoidosis with involvement of the nervous system. Report of nine cases. Arch Neurol Psychiatry 1954;71:554-75. 5Suchenwirth R, Dold V. Functional psychoses in sarcoidosis. Verh Dtsch ges Inn Med 1969;75:757-59. 6 Zerman W. Die meningoencephalitis Besnier-Boeck-Schuumann. Nervenartz http://jnnp.bmj.com/ 1952;23:43-52. 7Morehouse H, Danziger A. CT findings in intracranial neurosarcoid. Comput Tomogr 1980;4:267-70. 8Kumpe DA, Rao-Krishna CVG, Garcia JH, Heck AF. Intracranial neurosarcoidosis. J Comput Assist Tomogr 1979;3: 324-30. Babu VS, Eisen H, Pataki K. Sarcoidosis of the central nervous system. J Comput Assist Tomogr 1979;3: 396-7. on October 1, 2021 by guest. 0 Brooks J, Strickland MC, Williams JP, Vulpe M, Fowler FL. Computed tomography changes in neurosarcoidosis clearing with steroid treatment. J Comput Assist Tomogr 1979;3:398-9. Ho SU, Berenberg RA, Kwang KS, DalCanto MC. Sarcoid encephalopathy with diffuse.
Recommended publications
  • Consequences of Sarcoidosis

    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.
  • An Elderly Patient with Sarcoidosis Manifesting Panhypopituitarism with Central Diabetes Insipidus

    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].
  • Coexistence of Neurosarcoidosis and Multiple Sclerosis

    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).
  • An Epidemic of Acute Encephalitis in Young Children

    An Epidemic of Acute Encephalitis in Young Children

    Arch Dis Child: first published as 10.1136/adc.9.51.153 on 1 June 1934. Downloaded from AN EPIDEMIC OF ACUTE ENCEPHALITIS IN YOUNG CHILDREN BY AGNES R. MACGREGOR, M.B., F.R.C.P.E., AND W. S. CRAIG, B.Sc., M.D., M.R.C.P.E. (From the Departments of Pathology and Child Life and Health, Univer- sity of Edinburgh, and the Western General Hospital, Edinburgh.) This paper is concerned with a small outbreak of illness of an unusual nature occurring in the Children's Unit of the Western General Hospital, Edinburgh. In addition to the clinical interest of the cases, there are features of considerable pathological and epidemiological importance con- nected with the outbreak. Clinical Records. Case 1. I.M.S., female, aged 1 year 7 months, was admitted to the Western General Hospital in March, 1933, at the age of 1 year 3 months. - At this time http://adc.bmj.com/ she was noted as being slightly undersized but well nourished and the liver showed moderate enlargement. Prior to admission she had been treated elsewhere for gonococcal vaginitis: during the three succeeding months her health was good and progress uninterrupted, but a positive Wassermann reaction, present on admission, persisted. On the evening of July 22, 1933, the patient was noticed to be less active than usual and generally ' out of sorts ': her conditiQn remained unchanged throughout the rest of the day, and on the 23rd she was 'Ptill quieter and less responsive to all forms of attention and refused food. By the afternoon on October 2, 2021 by guest.
  • Neurosarcoidosis

    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].
  • Encephalitis Lethargica

    Encephalitis Lethargica

    flra/n (1987), 110, 19-33 ENCEPHALITIS LETHARGICA A REPORT OF FOUR RECENT CASES Downloaded from https://academic.oup.com/brain/article/110/1/19/273787 by guest on 01 October 2021 by R. s. HOWARD and A. J. LEES (From The National Hospital for Nervous Diseases, Queen Square, London) SUMMARY Four patients are described with an encephalitic illness identical to that described by von Economo. Electroencephalographic, evoked potential and autopsy data suggest that involvement of the cerebral cortex is more extensive than has been generally recognized. Serological tests and viral cultures failed to reveal the infectious agent but the presence of oligoclonal IgG banding in the cerebrospinal fluid in 3 of the patients during the acute phase of the illness would be in keeping with a viral aetiology. INTRODUCTION Accounts of febrile somnolent illnesses with residual apathy, ophthalmoplegia, chorea and weakness abound in the early literature. The Schlafkrankheit of 1580, Sydenham's febris comatosa of 1672-1675 in which hiccough was a prominent symptom, febre lethargica of 1695, coma somnolentium of 1780, Gerlier's vertige paralysante of 1887 and the dreaded Italian nona of 1889-1890, in which sleepiness, cranial nerve palsies and tremor occurred, are some examples of what may be a recur- ring plague caused by the same aetiological agent (Wilson, 1940; Sacks, 1982). Despite these historical forerunners, the sleeping sickness pandemic of 1916-1927 burst forth spontaneously in several different European cities unrecognized and then relentlessly spread around the world leaving an estimated half a million people dead or disabled. Constantin von Economo, however, relying in part on recollec- tions of his parents' descriptions of nona, was able to show that what appeared as a series of unrelated polymorphous outbreaks was in fact a disease caused by a single transmissible factor.
  • The Pathogenesis and Treatment of Optic Disc Swelling in Neurosarcoidosis a Unique Therapeutic Response to Infliximab

    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).
  • Postencephalitic Parkinsonism

    Postencephalitic Parkinsonism

    University of Nebraska Medical Center DigitalCommons@UNMC MD Theses Special Collections 5-1-1931 Postencephalitic parkinsonism H. Alva Blackstone University of Nebraska Medical Center This manuscript is historical in nature and may not reflect current medical research and practice. Search PubMed for current research. Follow this and additional works at: https://digitalcommons.unmc.edu/mdtheses Part of the Medical Education Commons Recommended Citation Blackstone, H. Alva, "Postencephalitic parkinsonism" (1931). MD Theses. 141. https://digitalcommons.unmc.edu/mdtheses/141 This Thesis is brought to you for free and open access by the Special Collections at DigitalCommons@UNMC. It has been accepted for inclusion in MD Theses by an authorized administrator of DigitalCommons@UNMC. For more information, please contact [email protected]. SENIOR THESIS H. Alva Blackstone. POSTENCEY.dAlI T1 C PAEK m30lT Idl:i. ,~ WITH CASE REPORTS 1931. POSTENCEPF.u!i.LITIC p~4.RKmSONrSIU This cond i tion may be differently spoken of as mesencepha­ litic Parkinsonism or as chronic encephalitis Ie thargica exhi bi ting a Parkinson's syndrome. It is also known as encephalitic or posten­ cephali tic paralys is agi taus. The Parkins onism of pos tencephalitis is considered generally as a sequel of aoute epidemio encephalitis. Later studies, clinically and pathologically, have given rise to the belief that a Parkinsonism follovdng encephalitis (acute epidemic) may not be a sequel, but a s;yrrptom syndrome due to chronic inflammatory changes vVhich are a part of the acute encephalitic entity. For the sake 0 f pas t studies of the disease, it shall be cons idered for the present as a sequel of acute epidemic enoephali tis vd th a pathology of degenerati on changes in the basal ~nglia.
  • Sarcoidosis Optic Neuropathy

    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.
  • Neurosarcoidosis: Clinical Manifestations, Investigation and Treatment

    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.
  • Neurosarcoidosis-Demonstration of Meningeal Disease by Gadolinium

    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.
  • Neurosarcoidosis Manifesting As Tremor of the Extremities and Severe Hypopituitarism —Case Report—

    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