A 53-year-old man was admitted to the Neurology Department of Farabi Hospital (Kermanshah, Iran) with walking inability and recognition disorder. Patient history showed that he had referred to hospital with vomiting, vertigo and headache 8 months before after consumption of two sachet of levamisole (for veterinary use) which each sachet contained 1.5 gram of levamisole hydrochloride. One month later, he had been stricken by progressive walking inability and recognition disorder up to administration to our hospital. The patient had not any previous illness.\nOn neurologic examination, he was confused and did not establish a good relationship with the bystander. On mental and cognitive power examinations, using the Mini Mental State Examination (MMSE), he earned 17 points (out of a total score of 30) with no signs of meningeal irritation (such as stiffness of neck). In addition, cranial nerves were normal. The power of limbs was within normal range but deep tendon reflexes in all of four limbs had increased in conjunction with extensor plantar response and reduced abdominal skin reflex. The patient had primitive reflexes including grasp, snout and palmomental. On cerebellar examination, finger to nose test and heel to shin was impaired and the gait was ataxic.\nOn the initial laboratory tests, complete blood count (CBC), blood sugar, urea, creatinine, and liver enzymes were normal. In cerebrospinal fluid analysis, color, pressure, cells, and sugar were normal but protein was increased (69 mg/dl). Oligoclonal band (OGB) was negative.\nOn electroencephalography (EEG), bilateral paroxysmal slow waves were seen. In visual evoked potential (VEP), there was a bilaterally prolonged P100 latency. Brain magnetic resonance imaging (MRI) showed mild cerebral atrophy and multiple focal lesions distributed in periventricular and cerebellar white matter and also in pons and midbrain (). These lesions had low-signal intensity on T1-weighted images and high-signal intensity in fluid-attenuated inversion recovery (FLAIR) view and T2-weighted images.\nFollowing clinical examinations and ruling out of other causes, including infectious and vascular causes, trauma and metabolic, central nervus system (CNS) vasculitis and paraneoplastic syndromes; and according to the patient's history, the patient was diagnosed as multifocal inflammatory leukoencephalopathy following levamisole consumption. The patient did not give consent for brain biopsy.\nThe patient was treated with intravenous methylprednisolone (500 mg) every 12 hours for 7 days followed by two weeks oral consumption of the prednisolone (50 mg/day) during hospitalization. No change was noticed in cerebellar signs and gait disorder but a brief improvement was seen in mentality and cognition after 1 and 3 months. After a month of treatment, MRI did not show a reduction or remission of the lesions. ()
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.