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 29-year-old man was referred to the emergency department (ED) due to severe headache and behavioral changes.\nThe symptoms had started 12 days before admission with the headache considered as migraine and treated by oral pain killers. The patient came back to the ED 2 days later with persisting headache and right-sided paresthesia. Brain computed tomography (CT) failed to reveal any abnormality, and the patient was sent home again with nonsteroidal anti-inflammatory drugs and tramadol.\nTwo days later, he developed severe behavioral and cognitive changes with incomprehensible vocalizations, anterograde amnesia, aggressiveness towards his family members, and agitation. Sleeping rhythm was inverted with alternating periods of apathy/somnolence and agitation.\nPatient's medical history was unremarkable, and he did not take any medication. Occasional use of tobacco and alcohol was reported. We learned from his father that he had started regular cocaine consumption 3 months ago but should have stopped about one week before admission.\nOn admission, the patient was awake, but disorientated in time and place, and did not properly answer to questions. There was no sign of lateralization and cerebellar tests were normal. Neck stiffness and photophobia were noted.\nRoutine laboratory investigations were unremarkable, as well as blood and urine toxicological screening. Repeated brain CT was also normal. Lumbar puncture showed a normal opening pressure, and cerebrospinal fluid (CSF) analysis revealed 7 mononuclear cells/mm3 and a high protein level at 224 mg/dL. The albumin quotient was highly increased (37.9, normal value <6.5 under 40 years of age), confirming, together with the further brain magnetic resonance imaging (MRI) findings, significant alteration of the blood-CSF barrier permeability. Microbiological and viral CSF tests were negative. No CSF-specific IgG oligoclonal bands were found. As infectious encephalitis was ruled out, antimicrobial and antiviral therapy was interrupted.\nWhile staying in the ICU, the patient had been less agitated but remained disoriented in time and space and had suffered from transcortical sensitive aphasia. Brain MRI was performed 14 days after symptom onset which showed ubiquitous small lesions within white matter displaying high signal intensity on diffusion-weighted images and water diffusivity restriction on apparent diffusion coefficient (ADC) maps. The radiologist initially considered the hypothesis of disseminated ischemic foci due to cocaine-related vasoconstriction (not illustrated). Brain MRI examination was repeated after 3 days and showed numerous additional punctate lesions involving the corpus callosum, together with abnormal meningeal enhancement and contrast agent leakage in the CSF (Fig. ).\nRetinal fluoroangiography revealed multiple segmental occlusions of the central retinal artery (Fig. ), and an audiogram showed bilateral but asymmetric sensorineural hearing loss, with a left predominance.\nAfter the diagnosis of SS, bolus corticosteroid therapy was initiated using methylprednisolone 1 g daily for 5 days, followed by tapering oral doses. Toxicological investigations were completed by hair analysis using ultra-performance liquid chromatography tandem mass spectrometry operating in positive electrospray mode (Quattro Premier, Waters, Milford, MA, USA). Hair samples were cut into six segments of 1 cm, and the presence of cocaine, levamisole, opioids, and several amphetamines was investigated. Only cocaine together with metabolites, levamisole, and tramadol were detected. Quantification was possible using deuterated analogues: cocaine-D3 for cocaine and norcocaine, benzoylecgonine-D8 for benzoylecgonine, methylecgonine-D3 for methylecgonine, 6-acetylmorphine-D6 for levamisole, and tramadol-D3 for tramadol. The following concentration ranges were found in the hair: cocaine (>5,000 pg/mg), benzoylecgonine (>5,000 pg/mg), methylecgonine (68–113 pg/mg), norcocaine (581 to >1,000 pg/mg), levamisole (73–139 pg/mg), and tramadol (1,378–2,155 pg/mg). The patient was transferred from our institution to a rehabilitation center 13 days after admission. Cognitive status progressively improved, but the patient remained dysexecutive and had features of frontal lobe syndrome. Five weeks after discharge, the patient presented with a subacute left-eye central scotoma and altitudinal visual field deficit while still on a daily oral dose of 16 mg methylprednisolone. New retinal branch artery occlusions were documented on fluoroangiography (Fig. ). However, brain MRI did not show additional microvascular ischemic lesions when compared to previous examination, and lepto-meningeal enhancement had significantly decreased (not shown). This relapse (or progression of the initial lesions) was again treated by repeated methylprednisolone pulse therapy followed by oral tapering. Immunosuppressive treatment with mycophenolate mofetil was started. At 10-month follow-up, the patient was asymptomatic. He resumed work and did not report any residual cognitive deficit, as confirmed by neuropsychological testing performed 3 months after the initial episode.