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 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. ()