Determination of Angiotensin-Converting Enzyme Levels in Cerebrospinal Fluid Is Not a Useful Test for the Diagnosis of Neurosarcoidosis

Dale, Jane C.; O’Brien, John F.

doi:10.1016/s0025-6196(11)65143-4

Cited by 65 publications

(23 citation statements)

References 4 publications

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“…OCBs are present in a minority of neurosarcoidosis patients. CSF ACE is normal in more than 50% of patients [114][115][116]. On brain MRI, neurosarcoidosis lesions commonly enhance with gadolinium and may involve the leptomeninges, which is not typically seen in MS, or manifest as discreet parenchymal lesions.…”

Section: Diagnosismentioning

confidence: 99%

A differential diagnosis of central nervous system demyelination: beyond multiple sclerosis

2011

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Although multiple sclerosis (MS) is the most common demyelinating disorder of the central nervous system (CNS), it lacks any definitive diagnostic test. Instead, diagnosis of MS primarily depends upon clinical criteria, supported by abnormalities characteristic of MS on para-clinical investigations including magnetic resonance imaging of the brain and spine, in the absence of an alternative explanation for underlying neurologic symptoms. While many of the potential disorders that may mimic MS in routine clinical practice are either extremely rare, or associated with specific and characteristic distinguishing diagnostic features, some inflammatory demyelinating disorders of the CNS may be particularly challenging to distinguish from MS, especially during initial presentation. In particular, acute disseminated encephalomyelitis, neuromyelitis optica, and idiopathic transverse myelitis may closely resemble MS, impeding prompt and accurate diagnosis. In this review, we describe the clinical features, diagnosis, pathology, and treatment of these other CNS demyelinating disorders. In addition, we review relevant features of other CNS inflammatory disorders that may mimic MS, including Sjögren's syndrome, systemic lupus erythematosus, Behçet's disease, and primary CNS vasculitis.

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Section: Diagnosismentioning

confidence: 99%

A differential diagnosis of central nervous system demyelination: beyond multiple sclerosis

2011

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“…However, CSF ACE levels were within normal limits in several individual cases [16,26,44,47,50]. In contrast, other investigators have recently pointed out that the CSF ACE level is not a very useful clue for the diagnosis of neurosarcoidosis [14]. Serum and CSF ACE levels may be used to control therapeutic efforts after the onset of steroid medication.…”

Section: Clinical Presentationmentioning

confidence: 99%

Neurosarcoidosis: a review of its intracranial manifestation

Nowak

Widenka

2001

Journal of Neurology

281

183

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Sarcoidosis is a multisystem disease of unknown cause and with a worldwide distribution. Involvement of the central nervous system occurs in a relatively small number of patients with sarcoidosis. Isolated neurosarcoidosis without signs of systemic disease is a rarity. Because of its non-specific clinical presentation and neuroradiological imaging characteristics, intracranial neurosarcoidosis remains a very difficult diagnosis, particularly in the absence of systemic signs of the disease. Intracranial neurosarcoidosis has a predilection for the basal leptomeninges commonly affecting the cranial nerves, but any part of the brain may be involved, resulting in a wide spectrum of clinical syndromes. Cranial nerve involvement is the most common single symptom. Intracranial sarcoid manifests as nodular or diffuse leptomeningeal thickening and extraor intra-axial parenchymatous lesions. Intracranial sarcoid may mimic various forms of meningitis, including carcinomatous and intracranial mass lesions such as meningioma, lymphoma and glioma, based on neuroradiological imaging. Magnetic resonance imaging is a very sensitive diagnostic tool for detecting intracranial abnormalities due to neurosarcoidosis. Lumbar puncture is useful in ruling out other neurological disorders, in particular infectious, but cerebrospinal fluid findings are not specific. Angiotensin-converting enzyme in serum and cerebrospinal fluid may be increased, decreased or normal. Therapy consists of immunosuppressive agents and should be initiated with corticosteroids. Other immunosuppressive drugs should be added in severe cases or after frequent recurrences. We review here all aspects of intracranial neurosarcoidosis from the clinical point of view, with special emphasis on presentation, diagnostic procedures, differential diagnostic considerations and treatment.

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“…However, many authorities now regard this test as non-specific and insensitive. CSF ACE may be raised in infections and malignancy, and appears unhelpful in any therapeutic decision making 28 . This is illustrated by one study of 32 patients with sarcoidosis, including 20 with NS: CSF ACE levels were raised in only 55% of patients with NS, in 5% of patients with sarcoidosis not apparently involving the nervous system, and in 13% of patients with other neurological diseases 29 .…”

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confidence: 99%

Neurosarcoidosis: Two case reports with multiple cranial nerve involvement and review of the literature

Mijajlović¹,

Mirkovic²,

Mihailović-Vučinić³

et al. 2014

Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub

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aBackground. Involvement of the central nervous system is registered in a relatively small number of patients with sarcoidosis. In this article we present two cases with various neurological symptoms that fulfill criteria for neurosarcoidosis (NS). In addition, we review the literature on NS with special attention to isolated cranial nerve involvement. Methods and Results. First patient: Neurological examination identified multiple cranial neuropathy, moderate rightsided hemiparesis, polyradiculoneuritis of the lower limbs and positive meningeal signs. Laboratory tests showed serum and cerebrospinal fluid (CSF) inflammatory abnormalities, with increased values of the angiotensin-converting enzyme (ACE). CSF analysis also showed presence of 9 oligoclonal IgG bands. Brain and spine magnetic resonance imaging (MRI) revealed diffuse meningopathy, and focal granulomatous lesion in the body of the L5 vertebra. Lung sarcoidosis was confirmed by additional diagnostic procedures. The patient was treated with Methylprednisolone and a tapering course of oral Prednisone, which reduced the pain in the back and legs and improved the strength of the right leg. However, the other neurological deficiencies remained. After confirming lung sarcoidosis, the patient received Methotrexate in addition to Prednisone but during the following 2 years the patient's condition progressively worsened and ended in death. Second patient: Neurological findings showed weakness of the right n. oculomotorius and the right n. trochlearis, as well as the right-side face weakness. We found raised level of the ACE in serum and CSF. Thorax high-definition computed tomography (HDCTT) showed ribbon-like domains of discrete changes in the pulmonary parenchyma. MRI of the brain showed multiple white matter lesions. This patient also received Methylprednisolone followed by Prednisone, and after two months, ocular motility normalized. Conclusion. The diagnosis of NS is always a challenge. For this rerason definitive diagnosis requires the exclusion of other causes of neuropathy. Multiple cranial neuropathies should always arouse suspicion of NS.

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Determination of Angiotensin-Converting Enzyme Levels in Cerebrospinal Fluid Is Not a Useful Test for the Diagnosis of Neurosarcoidosis

Cited by 65 publications

References 4 publications

A differential diagnosis of central nervous system demyelination: beyond multiple sclerosis

A differential diagnosis of central nervous system demyelination: beyond multiple sclerosis

Neurosarcoidosis: a review of its intracranial manifestation

Neurosarcoidosis: Two case reports with multiple cranial nerve involvement and review of the literature

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