A 25-month-old male with a history of mild reactive airway disease and sickle cell trait was noted by his mother to be breathing slowly and erratically 2 h after being laid down for a nap. Emergency medical services were called and paramedics encountered a pulseless, apneic patient. Cardiopulmonary resuscitation was immediately initiated. Return of spontaneous circulation was achieved in the ambulance on route to our institution’s emergency department.\nOn arrival, physical examination revealed an afebrile toddler, unresponsive to voice or noxious stimulation with agonal respirations, a blood oxygen saturation of 93% with bag-mask-assisted ventilation, and a blood pressure of 99/65 mmHg (mean arterial pressure 76 mmHg). Neurologic examination demonstrated 3 mm, non-reactive pupils and global hyperreflexia. Orotracheal intubation was performed emergently. Initial laboratory evaluation revealed normal electrolytes (Na+ 138 mEq/L, K+ 4.9 mEq/L, Cl− 104 mEq/L, Ionized Ca2+ 5.2 mEq/L), leukocyte count (11,100/mm3), venous blood gases (pH 7.37, PvCO2 44, PvO2 47, 25.7), and serum lactate (0.9 mmol/L). Upon further interrogation, the patient’s mother reported the presence of prescription opiates (extended release morphine) in the household, which she uses for pain control secondary to frequent sickle cell anemia crises. A total of two doses of naloxone (0.1 mg/kg i.v. push each) were administered, after which the patient regained pupillary reactivity and began moving all four extremities spontaneously. A urine toxicology panel was obtained, which was positive for morphine.\nA non-contrast head CT was obtained (Figure B), which revealed acute hydrocephalus and extensive, symmetric bilateral cerebellar hemispheric hypoattenuation consistent with severe cerebellar edema that resulted in compression of the fourth ventricle and displacement of the midbrain and pons superiorly. Cervicomedullary junction compression secondary to cerebellar tonsillar herniation was also noted. Neurosurgery was consulted and the patient was emergently taken to the operating room for the placement of an external ventricular drain, suboccipital craniectomy, and a C1 laminectomy. A significant portion of the edematous and ischemic cerebellar hemispheres and tonsils were resected bilaterally, thereby decompressing the posterior fossa and cervicomedullary junction.\nA brain MRI obtained on postoperative day 1 demonstrated cerebellar and cervicomedullary junction decompression, with extensive restricted diffusion in the remaining cerebellar parenchyma (Figure B). Multifocal areas of diffusion restriction were also noted bilaterally in the anterior cerebral artery-middle cerebral artery and middle cerebral artery-posterior cerebral artery watershed territories involving portions of the frontal, occipital, and temporal lobe white matter. An MRA/MR venogram demonstrated no craniocervical or intracranial arterial abnormalities or evidence of cortical or dural venous sinus thrombosis. The patient was extubated on postoperative day 5. Assessment at postoperative day 18 demonstrated an easily arousable child, with spontaneous eye opening, equally round and reactive pupils, and with purposeful movements of all four extremities.\nOutpatient follow-up at 29 months of age (3 months after presentation) demonstrated an active child speaking in full sentences. He was able to ambulate independently, albeit with a slightly widened base of support, and mild left lower extremity spasticity which did not limit his ability to climb or descend stairs with rail support.
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. ()