A 41-year-old woman presented to the emergency room of another hospital with recent confusion and impaired balance. She had been using cocaine daily for seven months. MRI FLAIR images showed multiple white matter signal changes in both cerebral hemispheres in a predominantly periventricular distribution (Fig. –) sparing the corpus callosum, cerebellum and brainstem. Restricted diffusion was present in many lesions (not shown). Subtle gadolinium enhancement was present in a few lesions (not shown). She was treated with supportive care for one week and then discharged with partial recovery. She resumed cocaine use and was then admitted to our centre six weeks after the previous hospital discharge because of worsening neurological symptoms. She was completely bedridden with inability to walk, incontinence, severely impaired communication skills, behavioural problems, cognitive impairment, and multifocal neurological deficits. MRI FLAIR images showed marked progression compared to the previous study. Confluent white matter abnormalities involved both cerebral hemispheres (Fig. –). There was subtle scattered restricted diffusion and no gadolinium enhancement. Urine testing was positive for cocaine, oxycodone, opiates, benzodiazepines, tramadol and citalopram, and negative for cannabinoids, amphetamine and methadone. Other investigations included the following normal or negative results: complete blood count, electrolytes, glucose, liver enzymes, thyroid stimulating hormone, T3, T4, creatinine kinase, angiotensin converting enzyme, HIV, Hepatitis A, B, and C screens, Lyme serology, syphilis screen, blood screen for salicylates, acetaminophen and ethanol. The serum creatinine was elevated (137 micromoles/l) as was the urea (7.6 millimoles/l). Serum albumin was low (29 g/l). The erythrocyte sedimentation rate ranged from 22 to 64 on multiple samplings, C-reactive protein was elevated at 11 mg/l while antinuclear antibodies, pANCA, cANCA, C3, C4, NMDA receptor and anti-thyroid peroxidase antibodies were normal. The vitamin B12 level was low at 166 picomole/l (normal >180). Copper, zinc and ceruloplasmin levels were normal. CSF protein was slightly elevated at 0.48 g/l. The glucose, chloride and cell count (4 × 106/l) were normal. No CSF acid fast bacilli were seen and cultures for bacteria and fungi were negative. CSF PCR for CMV and HSV1–2 were negative. She was treated with intravenous methylprednisolone, 1000 mg/day for five days, and plasma exchange. She showed substantial, albeit partial, recovery soon thereafter which continued over the course of several months. At four months follow-up multiple domains of cognitive function were improved but she had some residual deficits in attention, calculation, and visuospatial function. She was independent with respect to walking and most activities of daily living. Follow-up MRI at that time showed improvement of the subcortical and periventricular signal abnormalities and no new lesions. Similarly, no new lesions were seen on MRI 13 months after presentation (not shown).
A 41-years-old-man addicted to chronic cocaine and alcohol abuse was admitted to our emergency department in a stuporous state and with urinary incontinence. In the previous 15 days he had developed dysarthria, mutism with a prominent drowsiness and psychomotor disturbances. A brain MRI, abdomen and chest computed tomography (CT) were performed in another hospital. MRI brain scan showed multiple roundish lesions in the periventricular cerebral white matter.\nOn admission, laboratory tests were within normal limits, and the virological screening together with the Quantiferon, HIV and Treponemal tests was negative. Only C-reactive protein value resulted higher than normal (1.18 mg/L).\nA cerebrospinal fluid (CSF) examination revealed a high albumin level, no oligoclonal bands and a normal immunoglobulin G index. The electroencephalogram (EEG) recorded low α and θ waves.\nUrine toxicologic screening test detected low level of cocaine and it was negative for benzodiazepines, barbiturates, oxycodone, amphetamine, methadone and cannabinoids. The patient’s urine samples were not tested for levamisole. No myelin-oligodendrocyte-glycoprotein (MOG) or aquaporin antibodies 4 were documented.\nFirst, the patient underwent a brain CT study which detected multiple hypointense circular lesions in the bihemispheric white matter surrounded by edema.\nBrain MRI revealed multiple well-circumscribed concentric lamellar lesions in the bihemispheric white matter involving the corpus callosum,; these lesions showed alternate bands of iso-/hypointensity on T1-weighted images and alternate hypo-/hyperintensity on T2-weighted/fluid attenuation inversion recovery (FLAIR) images and presented with a rim of restricted diffusion on diffusion-weighted imaging (DWI; b = 1000), more obvious on the edge of the lesions. Apparent diffusion coefficient (ADC) map confirms alternate bands of diffusion restriction, displaying low signal intensity.\nAfter gadolinium intravenous administration, all the lesions demonstrated an “onion-like” patchy enhancement on post-contrast T1-weighted images.\nDouble inversion recovery (DIR) sequences showed more detailed lamellar structure of the lesions than FLAIR images, thanks to high sensitivity in juxta and subcortical lesions detection.\nMR spectroscopy revealed elevated choline and lactate peaks and decreased N-acetylaspartate peak ().\nMRI of the spine showed no evidence of demyelinating lesions. Stereotactic biopsy obtained from frontal right paraventricular lesion showed gliosis and many swollen reactive eosinophilic astrocytes with perivascular cuffing. Axonal stain showed long stretches of intact axon. In addition, perivascular and periventricular macrophages were found (). These pathologic findings supported a possible inflammatory/immune-mediated etiology.\nThe patient was treated with high-dose oral methylprednisolone for 15 consecutive days, after which his clinical symptoms partially improved.