Книги по МРТ КТ на английском языке / MR Imaging in White Matter Diseases of the Brain and Spinal Cord - K Sartor Massimo Filippi Nicola De Stefano Vincent Dou

sivity of protons in tissue. In highly structured tissue, such as the brain, movement of protons is limited by multiple barriers, such as the myelin sheaths. In diseases that are associated with loss of tissue structure, such as demyelinative diseases, diffusivity of the brain tissue increases, which is reflected in elevated ADC values. On the other hand, abnormally decreased ADC values can also be encountered. Protons located in the interstitium experience more diffusivity than those located within the cells. Cytotoxic edema, which accompanies the early stage of a brain infarct, is characterized by an increase of the intracellular compartment at the expense of the extracellular compartment. Consequently, this gives rise to decreased diffusivity and ADC values.

Similar to MTR histograms, histograms can also be generated based on DWI studies. Bosma et al. performed DWI in primary NPSLE patients and healthy controls without significant abnormalities on conventional brain MRI (Bosma et al. 2003). The ADC histograms of NPSLE patients differed form those of controls. The histogram peak was lower and wider, and mean ADC values were higher in NPSLE patients, reflecting loss of uniformity and increase in brain diffusivity. In the same study, no abnormalities suggestive of cytotoxic edema were found. The observations from this study support the data from studies using MTR and MRS, in that in NPSLE structural damage is much more widespread than expected from conventional MRI.

Moritani et al. observed areas of hyperintensity on DWI and increased ADC representing vasogenic edema in a series of 20 SLE patients with neurological abnormalities. Some of these lesions resolved partially and some completely following treatment. In four patients, a hyperintense focus on DWI associated with a decreased ADC representing an acute infarction was observed (Moritani et al. 2001).

21.4.5

Perfusion-Weighted Imaging

Tracer bolus passage studies, such as perfusionweighted imaging (PWI), allow mapping CBF without radiation exposure (Sibbitt et al. 1999). Borrelli et al. found 13 hypoperfused areas in 10 of 20 NPSLE patients using PWI; however,PWI showed fewer hypoperfused areas than did SPECT (43 hypoperfused areas in 17 of 20 patients; Borrelli et al. 2003). So far, PWI has been used little in NPSLE research; however, it has been suggested that in the diagnostic work-up PWI can exclude a CNS vasculitis (Yuh et al. 1999b).

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CONTENTS

22.1Introduction 331

22.2.1 Systemic Vasculitides 331

22.2.1.1Behçet’s Disease 331

22.2.1.2Sjögren Syndrome 334

22.2.1.3Polyarteritis Nodosa 336

22.2.4Neurosarcoidosis 338

22.3Conclusions 340 References 340

22.1 Introduction

Many non-multiple sclerosis (MS) inflammatory diseases of the central nervous system (CNS) can cause a multifocal white matter damage of the brain similar to that of MS. In many of them, however, the damage is not confined to the white matter. Two of the latter conditions, namely systemic lupus erythematosus (SLE) and primary angiitis of the CNS,are dealt with in other chapters herein (Van Buchem: neuro-SLE; Okuda: primary angiitis of the CNS). Herein, we shall review the conventional MR imaging features of the CNS (brain, optic nerve and spinal cord) lesions besides white matter in some of the systemic vasculitis–including Behçet’s disease, Sjögren syndrome and polyarteritis nodosa–which can help in the differential diagnoses of the white matter multifocal lesions (Miller et al. 1987). Other systemic angiitis including rheumatoid arthritis (Bekkelund et al. 1995), giant cell arteritis, and Wegener disease, albeit capable of producing brain damage, usually do not determine white matter

M. Mascalchi, MD

Professor, Sezione di Radiodiagnostica, Dipartimento di Fisiopatologia Clinica, Università di Firenze, Viale Morgagni, 85, 50134 Firenze, Italy

F. Salvi, MD

Sezione di Radiodiagnostica, Dipartimento di Fisiopatologia Clinica, Università di Firenze,Viale Morgagni, 85, 50134 Firenze, Italy

changes and, consequently, will not be addressed. In addition, the CNS damage associated with vasculitis due to the use of illicit drugs such as amphetamines will not be reviewed. On the other hand, the MR findings that can be observed besides the white matter damage in antiphospholipid antibody syndrome will be reviewed. Finally, after a brief account on postinfective angiitis, we shall review the wide spectrum of lesions beyond the white matter in sarcoidosis.

Conventional brain MR imaging is here considered the usual protocol that includes T1-weighted, proton density-weighted and T2-weighted (with and without inversion recovery RF pulse to attenuate the CSF signal) sequences, T1-weighted images after administration of intravenous gadolinium chelates (at equilibrium) and MR angiography. In addition, the MR imaging findings in the optic nerve and spinal cord will refer to the usual techniques, namely T1-weighted and fat-suppressed T2-weighted images and contrastenhanced, fat-suppressed T1-weighted sequences for the optic nerve, and T1-weighted, T2-weighted and contrast-enhanced, T1-weighted sequences for the spinal cord.

22.2.1

Systemic Vasculitides

22.2.1.1 Behçet’s Disease

Behçet’s disease (BD) is a systemic inflammatory disease of unknown aetiology that typically affects young adults in Japan,the Middle East and the Mediterranean area (Inaba 1989) along the ancient Silk Route. Since there is no specific laboratory or pathological feature, the diagnosis of BD rests entirely on clinical findings and should satisfy the criteria fixed in 1989 by an international study group (Lancet).These include recurrent oral ulcerations, plus at least two of the following four features: recurrent genital ulcerations, uveitis, positive pathergy test, and one of the skin lesions among erythema nodosum, pseudofolliculitis and papulopustu-

lar lesions. Neurological complications of BD occur in 10–49% of cases, usually after other manifestations of the disease (Inaba 1989).

Brain

The most common MRI findings in BD are focal white matter and grey matter lesions in the cerebral hemispheres and brainstem that can enhance after intravenous contrast administration, when examined in the acute phase (Kocer et al. 1999; Cellerini et al. 2003). A predilection was noted for the mesodiencephalic junction, with a distribution pattern similar to that of the intra-axial veins, suggesting that in BD the vasculitis is predominantly perivenular (Kocer et al. 1999). In some instances, the mesodiencephalic lesion may present as a large pseudo-tumoral lesion that rapidly resolves after corticosteroids (Kermode

et al. 1989) (Fig. 22.1). In a minority of patients intraparenchymal haemorrhagic lesions can be detected (Al Kawi 1991; Kocer et al. 1999), possibly reflecting a more necrotizing type of vasculitis, and these appear as hypointense areas due to deoxyhaemoglobin in the acute phase and due to haemosiderin in the chronic phase, when they are combined with overt atrophy (Fig. 22.2).

Thrombosis of the intracranial dural sinuses is another neurological presentation of BD and was emphasized as a common occurrence in one series (Wechsler et al. 1993). However, this was not confirmed in other series (Kocer et al. 1999; Cellerini et al. 2003). Interestingly, intracranial dural thrombosis generally affects younger individuals and does not occur in BD patients with multifocal brain lesions, and vice versa (Akman-Demir et al. 1999). The MRI features are similar to those in idiopathic intracra-

nial venous thrombosis (Condor and Jarosz 2002) (Fig. 22.3) and include intra-parenchymal haemorrhages with a lobar distribution, haemorrhagic infarcts in areas not corresponding to arterial territories, and signal changes in the thrombosed sinuses that are different depending on the time elapsed between thrombosis and imaging, the MR sequence and the field strength of the magnet. Combination of MR imaging and MR angiography is always recommended in the case of suspicion of dural sinus thrombosis, especially in the acute phase, when the thrombus may exhibit low signal in T2-weighted images due to deoxyhaemoglobin formation and hence be indistinguishable from the normal flowrelated signal void.

Optic Nerve

BD can affect the optic nerve and represent the first clinical evidence of the disease (Salvi et al. 1999). The MR imaging features are similar to those of MS and other secondary vasculitis affecting the optic nerve (Sklal et al.1996) and are better appreciated with fat-suppressed

T2-weighted images. They consist of increased signal intensity of one or both optic nerves (Fig. 22.4) and of contrast enhancement if the examination is performed in the acute phase (Kocer et al. 1999).

Spinal Cord

The spinal cord is involved in less than 20% of patients with BD and clinical signs of neurological involvement (Shakir et al. 1990). The cervical and thoracic segments can be affected, and MRI shows extensive lesions that exhibit contrast enhancement in the acute phase (Fig. 22.5) and almost completely resolve after steroid treatment (Mascalchi et al. 1998a; Kocer 1999).

22.2.1.2

Sjögren Syndrome

Primary Sjögren syndrome (SS) is most frequently a connective tissue disease (Lafitte 2002), and its diagnosis rests on the clinical findings of xerostomia and xerophthalmia, positive lacrimal or minor

d g

Fig. 22.4a–e. Behçet’s disease. Spinal cord damage in a 35-year-old woman. Sagittal T2-weighted image at presentation (a) shows an irregular, sharply defined area of increased signal in the spinal cord from T6 to T8. Axial T2-weighted images (b, c) show increased signal of the left half of the cord at T7 (b) and of the entire crosssection of the cord except the posterior columns at T7–T8 (c). Sagittal T1-weighted image after intravenous administration of a gadolinium chelate (d) shows moderate patchy enhancement of the lesion. Sagittal T2-weighted image (e) 5 months after clinical onset and treatment with corticosteroids shows a normal spinal cord. Reprinted from Mascalchi et al. 1998a

salivary gland biopsy and high titres of antinuclear and anti-SSA (Ro) and anti-SSB (La) antibodies. Neurological complications can occur in up to one-third of patients with primary SS, sometimes representing the clinical onset of the disease. Although peripheral nervous system involvement predominates (Lafitte et al. 2001), brain, optic nerve and spinal cord damage may be demonstrated by MRI.

Brain

Brain lesions in primary SS consist of nonspecific,multifocal white matter lesions that can be observed in patients with focal neurological deficit, psychiatric or

cognitive dysfunction alone, or in absence of any clinical sign or symptom of CNS dysfunction (Alexander et al. 1988; Pierrot et al.1993; Lafitte 2002). No other brain lesion besides the multifocal white matter changes have so far been documented with MRI.

Optic Nerve

In primary SS, optic nerve involvement is distinctly rare and usually symptomatic (Tesar et al. 1992; Kadota et al. 2002). In the only reported case (Kadota et al. 2002), the MRI features were indistinguishable from those of other optic nerve vasculitis (Sklar et al. 1996)