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CNS demyelinating lesions have been reported in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). There are no studies of cord atrophy in CIDP. Ten patients with CIDP underwent brain and spinal cord MRI to investigate CNS demyelination and cord atrophy. No CNS demyelination was found, but the mean cervical cord area was significantly smaller in CIDP patients vs control subjects. Spinal cord atrophy may be related to degeneration secondary to axonal loss.

Background: MRI findings influence the risk of patients with optic neuritis (ON) developing clinically definite (CD) multiple sclerosis (MS) but their influence on future disability is less clear. Objective: To investigate in patients with ON the influence of lesion number, location and activity, and non-lesion MRI measures obtained on early scans on disability. Methods: A total of 106 of 143 prospectively recruited patients with ON had reached a scheduled 5-year follow-up, of whom 100 were evaluated clinically. Lesion number, location, and activity measures were analyzed at baseline (within 3 months of onset) and lesion activity measures were studied at 3-month follow-up. Brain atrophy, magnetization transfer ratio, and spectroscopy measures were also analyzed. Ordinal logistic regression assessed the association between early MRI findings and subsequent disability. Results: At median 6 years follow-up, 48% had converted to CDMS and 52% remained with clinically isolated syndrome (median Expanded Disability Status Scale 2 and 1). In the final models, both the presence and the number of spinal cord lesions at baseline (odds ratios [OR] 3.30, 1.94) and new T2 lesions at follow-up (OR 7.12, 2.06) were significant independent predictors of higher disability. Disability was also predicted by the presence at baseline of gadolinium-enhancing lesions (OR 2.78) and number of infratentorial lesions (OR 1.82). Only spinal cord lesions predicted disability in patients converting to CDMS. Conclusion: Spinal cord, infratentorial, and gadolinium lesions within 3 months of optic neuritis onset and new T2 lesions after 3 months predicted disability after 6 years; only spinal cord lesions were predictive of disability in those developing clinically definite multiple sclerosis. BPF = brain parenchymal fraction; CD = clinically definite; CI = confidence interval; CIS = clinically isolated syndrome; EDSS = Expanded Disability Status Scale; FOV = field of view; Gd = gadolinium; GM = gray matter; GMF = gray matter parenchymal fraction; IQR = interquartile range; LR = likelihood ratio; MS = multiple sclerosis; MTI = magnetization transfer imaging; MTR = MT ratio; NAGM = normal-appearing gray matter; NAWM = normal-appearing white matter; ON = optic neuritis; OR = odds ratio; PRESS = point resolved spectroscopy; RRMS = relapsing remitting MS; SPMS = secondary progressive MS; TE = echo time; TR = repetition time; WM = white matter; WMF = white matter parenchymal fraction.

Objectives: To investigate ventricular enlargement (VE) over 1 year at three different stages of multiple sclerosis (MS). Methods: A semi-automated technique for measuring VE was applied to MRI scans in 26 patients with clinically isolated syndromes (CIS) suggestive of MS, 30 with early relapse-onset MS of 1 year duration, 41 with established relapsing remitting (RR) MS, and 23 with secondary progressive (SP) MS. Results: VE at 1 year was seen in early MS (median increase 0.3 mL [ p = 0.003]), RRMS (median increase 0.5 mL [ p = 0.001]), and SPMS (median increase 1.1 mL [ p = 0.001]). Allowing for age there was more VE in the SPMS group ( p = 0.005). No VE was observed in the CIS only group (median decrease −0.001 mL [ p = 0.829]). Significant increases in T2 and T1 hypointense lesion load volume were seen in all MS subgroups: there were no differences between the groups in T2 volume increase but there was a larger increase in T1 hypointense lesion volume in the SPMS group compared with early RRMS. Conclusions: Ventricular enlargement is a sensitive measure of progressive cerebral atrophy that is seen at all stages of multiple sclerosis (MS) and is more marked in secondary progressive than relapsing remitting MS.

Segmented normal-appearing brain tissue (NABT) was investigated in 40 patients with a recent onset and 13 patients with a remote onset of a clinically isolated syndrome (CIS) using magnetization transfer ratio (MTR) histograms. Abnormalities were present in patients with a high risk for MS (recent onset and T2-weighted lesions present) and in those with a low risk for relapse (recent onset without T2-weighted lesions). Similar mild NABT abnormality was present with CIS and no further disease activity 14 years later. NABT MTR abnormality in CIS may indicate susceptibility to demyelination but not to disease progression.

Treatment of acute optic neuritis with steroids has been shown to hasten visual recovery without affecting the final degree of recovery. However, MRI-clinical studies indicate that patients with long optic nerve lesions, particularly those that involve the nerve within the optic canal, may have a worse prognosis for recovery of vision. Partly because such lesions could lead to swelling and subsequent ischemic optic nerve damage, steroids could have a selective beneficial effect on this subgroup of patients. The present randomized trial was designed to test this possibility. Sixty-six patients with acute optic neuritis received IV saline or IV methylprednisolone. The clinical, psychophysical, electrophysiologic, and MRI outcomes were assessed after 6 months. Patients with short lesions presented earlier than those with long lesions (involving three or more 5-mm-thick slices of any part of the optic nerve, as well as its intracanalicular portion), and lesion length was significantly less in patients presenting within a week of onset of symptoms. Lesions also tended to lengthen during follow-up in individual patients. Treatment did not limit lesion length in either the long or short lesion subgroup and had no significant effect on final visual outcome. We conclude that steroids do not improve visual outcome or lesion length in patients with acute optic neuritis.

Paraneoplastic encephalomyelitis/paraneoplastic sensory neuronopathy (PEM/PSN) associated with small cell lung cancer is characterized by high serum and CSF titers of anti-neuronal (anti-Hu) antibodies and by intrathecal synthesis of anti-Hu IgG. A pathologic role for the anti-Hu antibodies in PEM/PSN is further suggested by reported intraneuronal accumulation of the antibodies in the nervous system of PEM/PSN patients at autopsy. We immunized SJL/J mice, Lewis rats, and Hartley guinea pigs with purified recombinant HuD fusion protein. In spite of high-titer anti-HuD antibodies, neurologic and pathologic examination of the animals was normal. Apparent uptake of purified IgG by neurons in the brain proved to be artifactual.

Objective: To apply multisequence MRI techniques to patients with clinically isolated syndromes, to document the pattern and frequency of abnormalities at baseline and early follow-up, and to determine their predictive values for the early development of clinical MS. Background: Disseminated lesions on T2-weighted brain MRI confer an increased risk of progression to clinically definite MS. Newer MRI techniques increase detection of lesions in both brain and spinal cord, and clarify further their pathology. The predictive value of such techniques for the development of clinical MS needs to be defined. Methods: Brain and spinal MRI were performed on 60 patients after their first demyelinating event. A total of 50 patients were followed for 1 year, and 49 underwent repeat brain MRI 3 months after the initial scan. Results: At baseline, 73% of patients had lesions on T2-weighted fast spin-echo (FSE) brain images and 42% had asymptomatic spinal cord lesions. Fast fluid-attenuated inversion-recovery brain did not improve detection of brain lesions. Repeat brain MRI demonstrated new FSE lesions in 43% of patients. After 1 year, 26% of patients developed MS. The MRI features that provided the best combination of sensitivity and specificity for the development of MS were the presence of new FSE lesions at follow-up and enhancing lesions at baseline. The frequency of developing clinical MS was higher for those with both brain and spinal cord lesions at baseline (48%) than brain lesions alone (18%). Conclusions: The combination of baseline MRI abnormalities and new lesions at follow-up, indicating dissemination in space and time, was associated with a high sensitivity and specificity for the early development of clinical MS. These data suggest a potential role for new diagnostic criteria for MS based on early MRI activity. Such criteria may be useful in selecting patients for therapeutic trials at this early clinical stage.

We have studied 18 patients with relapsing-remitting multiple sclerosis (MS) who had symptomatic visual field defects due to retrochiasmal lesions. In 17, the lesion responsible was identified by magnetic resonance imaging (MRI), computed x-ray tomography (CT), or both. The lesion responsible involved the posterior optic radiations in eight cases, the optic tract and lateral geniculate nucleus in six, and the posterior limb of the internal capsule in three. The prognosis for recovery of the field defect was good; complete recovery occurred in 14 patients, and only two showed no recovery at all. The striking characteristic of the lesions was that most were unusually large; indeed, many were detectable on CT as well as MRI. Half-field asymmetries of either amplitude or latency of the visual evoked potentials (VEPs), consistent with a postchiasmal lesion, were present in only five out of 13 patients acutely. In only three of these did the abnormality persist at follow-up. We conclude that only large postchiasmal lesions are likely to cause symptomatic homonymous field defects in MS, usually characterized by rapid recovery. Hemifield VEPs have a low sensitivity for the detection of postchiasmal as compared with prechiasmal abnormalities.

We describe a case of Behcet's disease with a slowly enlarging midbrain mass on magnetic resonance imaging, which resolved after 4 months of oral steroids.