Karen Black

Reversible occipital-parietal encephalopathy syndrome in an HIV-infected child

A reversible syndrome of headache, altered mental status, seizures, and cerebral visual abnormalities has been described in adults and children with hypertension, eclampsia, treatment with cyclosporin after organ transplantation, and interferon therapy.1-5 Neuroradiologic studies suggest predominantly posterior white matter abnormalities. Therefore, the syndrome has been referred to as reversible posterior leukoencephalopathy (RPL). We present a child with AIDS and RPL. We suggest that the brain is affected in a more extensive way and that both gray and white matter are involved. Case report. We report a girl with perinatal HIV infection untreated with antiretroviral medications until her first admission at the age of 12 years with Pneumocystic carinii pneumonia and disseminated mycobacterium avium intracellularae. She was severely immunosuppressed (CD4 = 1%, absolute 10, viral load 28,890 RNA copies/mL). Neurologic, cognitive, and ophthalmologic examinations were normal. She was doing well academically. Brain CT demonstrated diffuse atrophy. Antiretroviral therapy was begun, with improvement of CD4 values and reduction in viral load to …

CHILDHOOD AIDS, VARICELLA ZOSTER, AND CEREBRAL VASCULOPATHY

Case Report An 8-year-old girl presented to North Shore University Hospital with new onset of left third nerve palsy and a macular rash on her left cheek and around the left eye. She had a history of vertically acquired human immunodeficiency virus (HI~-1 infection, met clinical criteria for AIDS, and was treated with zidovudine and 2;3’-dideoxyinosine. Investigation revealed no evidence of toxoplasmosis, cytomegalovirus, Mycobacterium avium intracellulare,

Neonatal cerebral arterial thrombosis: protein C deficiency.

venous thrombosis: An unrecognized cause of transient seizures or lethargy. Ann Neurol 1992;32:51-56. 7. Shevell MI, Silver K, O’Gorman AM, et al: Neonatal dural sinus thrombosis. Pediatr Neurol 1989;5:161-165. 8. Wong VK, LeMesurier J, Franceschini R, et al: Cerebral venous thrombosis as a cause of neonatal seizures. Ped atr Neurol 1987;3:325-327. 9. Higashida RT, Helmer E, Van Halbach V, Hieshima GB: Direct thrombolytic therapy for superior sagittal sinus thrombosis. AJNR Am J Neuroradiol 1989;10:S4-S6. 10. Volpe JJ: Neurology of the Newborn, 3rd ed. Philadelphia, WB Saunders, 1995.

Venous sinus thrombosis as a cause of parenchymal and intraventricular hemorrhage in the full-term neonate

A case of parenchymal and intraventricular hemorrhage in a full-term neonate is reported. The underlying cause in our patient is thought to be related to cerebral sinovenous occlusive disease secondary to Protein C deficiency, a rare coagulopathy.

Anterior Thoracic Extradural Hematoma in a 5-Year-Old Child

Four hours following a motor vehicle accident, a 5-year-old girl developed a complete motor and sensory paraplegia below the sixth thoracic level (T6). The plain X-rays and MRI scan identified nondisplaced compression fractures of the T3-T6 vertebrae, associated with an anterior T3-T5 epidural hematoma. Following an emergency T3-T6 laminectomy, the sensory findings disappeared, while the motor deficit took 2 weeks to resolve. This case report emphasizes the value of the MRI scan in assessing pediatric spinal trauma, while underscoring the unique characteristics of acute epidural spinal hematomas.

Prevalence and Risk Factors for Paroxysmal Atrial Fibrillation and Flutter Detection after Cryptogenic Ischemic Stroke

INTRODUCTION Long-term cardiac monitoring with implantable loop recorders (ILRs) has revealed occult paroxysmal atrial fibrillation and flutter (PAF) in a substantial minority of cryptogenic ischemic stroke (CIS) patients. Herein, we aim to define the prevalence, clinical relevance, and risk factors for PAF detection following early poststroke ILR implantation. MATERIALS AND METHODS A retrospective study of CIS patients (n = 100, mean age 65.8 years; 52.5% female) who underwent ILR insertion during, or soon after, index stroke admission. Patients were prospectively followed by the study cardiac electrophysiologist who confirmed the PAF diagnosis. Univariate and multivariate analyses compared clinical, laboratory, cardiac, and imaging variables between PAF patients and non-PAF patients. RESULTS PAF was detected in 31 of 100 (31%) CIS patients, and anticoagulation was initiated in almost all (30 of 31, 96.8%). Factors associated with PAF detection include older age (mean [year] 72.9 versus 62.9; P = .003), white race (odds ratio [OR], 4.5; confidence interval [CI], 1.8-10.8; P = .001), prolonged PR interval (PR > 175 ms; OR, 3.3; CI, 1.2-9.4; P = .022), larger left atrial (LA) diameter (mean [cm] 3.7 versus 3.5; P = .044) and LA volume index (mean [cc/m2]; 30.6 versus 24.2; P = .014), and lower hemoglobin (Hb)A1c (mean [%] 6.0 versus 6.4; P = .036). Controlling for age, obesity (body mass index > 30 kg/m2; OR, 1.2; CI, 1.1-1.4; P = .033) was independently associated with PAF detection. DISCUSSION PAF was detected with high prevalence following early postcryptogenic stroke ILR implantation and resulted in significant management changes. Older age, increased PR interval, LA enlargement, and lower HbA1c are significantly associated with PAF detection. Controlling for age, obesity is an independent risk factor. A larger prospective study is warranted to confirm these findings.

Transforaminal lumbar puncture for intrathecal nusinersen administration: Noteworthy Cases

Nusinersen is the first effective treatment for spinal muscular atrophy (SMA). However, intrathecal administration of the drug presents challenges in SMA patients who have undergone spinal surgery for rod placement or fusion. Virtually all adult patients with type 2 SMA have had spinal surgery by adolescence as standard of care for scoliosis. A posterior interlaminar approach to lumbar puncture is often not possible in these patients, many of whom have completely fused spines. Fluoroscopic guidance may be complicated by the profound osteopenia often present. Options for accessing the intrathecal space include lumbar laminectomy with or without lumbar indwelling catheter placement and Ommaya reservoir placement. However, these approaches involve risks from factors such as general anesthesia and potential infection. SMA patients who have had spinal surgery have, in our experience, open and accessible neural foramina that can be easily visualized with computerized tomography (CT). We report a transforaminal lumbar approach for administration of nusinersen in adult SMA patients who have had complicated spinal surgery. Four patients aged 21–38 years with SMA type 2 and 1 patient aged 29 with SMA type 3 were evaluated for intrathecal nusinersen treatment. The type 2 patients had lower extremity weakness and inability to stand or walk, and SMA had been diagnosed by age 2 years. The type 3 patient could walk short distances with assistance, and SMA had been diagnosed at age 3 years. All patients required power wheelchairs for mobility and assistance for activities of daily living but had functional use of their hands and did not require ventilatory assistance, except for 1 who used noninvasive ventilation at night. Intrathecal nusinersen injection by lumbar puncture was attempted on our first patient by using a posterior interlaminar approach under fluoroscopic guidance but was unsuccessful because of prior spinal rod placement and severe osteopenia. A transforaminal approach was then used under CT guidance and was successful. For the procedure, the patients were placed in left or right lateral decubitus position and prepared with local anesthesia under sterile conditions. The subarachnoid space was accessed from an oblique trajectory (Fig. 1) at the L2-L3 foramen (L3-L4 in 1 patient) by using a 22gauge Chiba needle. Five cubic centimeters (12 mg) of nusinersen was injected into the intrathecal space after cerebrospinal fluid (CSF) flow had been demonstrated, and 5 cc of CSF was decanted. Three of the patients tolerated each procedure without complications and have completed 4 loading doses uneventfully. The fourth patient experienced a severe headache for 4 days after her first injection, and, subsequently, we were able to identify access at the L5-S1 interspace using a traditional posterior approach with CT guidance. The fifth patient has just had her first of 4 loading doses and did well. All patients were treated by an interventional neuroradiologist, with a neurologist present. These procedures were performed as outpatient procedures at our hospital radiology department, and patients were observed (without monitoring vital signs) for 1 h before discharge home. One patient requested conscious sedation (fentanyl and propofol) for her last of 4 loading doses because of pain she had experienced during the procedures and was attended to by an anesthesiologist. The other 4 patients reported minimal pain or discomfort during each procedure. Potential complications from this approach include postpuncture headache that may not be amenable to treatment by blood patch and radiculopathy due to the close proximity of the spinal nerve to the needle within the foramen. We have not seen radicular pain after any of the procedures to date and have had only 1 patient with a postprocedure headache, which resolved with bed rest and oral hydration. Peritoneal and retroperitoneal structures such as the kidneys and bowel are potentially in the path of the spinal needle. With careful CT scrutiny of the surrounding anatomy during each procedure and by tilting the patient slightly prone (to move peritoneal organs away from the needle trajectory), we have avoided inadvertent puncture of these organs. We are not aware of any data comparing interlaminar to transforaminal CT-guided lumbar puncture; however, we have used this approach (without thecal puncture) for periradicular epidural steroid injections without major complications. Researchers described use of this method for periradicular injections in 2 series of 143 and 231 patients and reported no major complications, although we view these reports cautiously because the thecal sac was not punctured in these studies. A transforaminal approach has been recently reported in a pediatric SMA cohort, and it is unclear whether smaller foramina in pediatric patients puts them at higher risk of complications. The average radiation exposure with CT guidance with the transforaminal method is comparable to a standard abdominal/pelvic CT examination at 9.95 mSV per treatment, as calculated from the mean radiation exposure of 10 treatments across 3 patients with an established milligray to millisieverts conversion factor of 0.018 for interventional abdominal/pelvic CT. The patient radiation dose from CT-guided injections is more than the exposure dose from fluoroscopy-guided lumbar interlaminar injections at our institution, which averages to 0.39 mSV when calculated across all fluoroscopy-guided lumbar interlaminar injections during the same period. However, as discussed above, repeated fluoroscopy-guided injections have been attempted in our patients without success because of spinal fusion and osteopenia, whether attempting a posterior or oblique approach. We believe that the small risk of the higher radiation dose of 4 abdominal/pelvic CT examinations (4 loading doses in 2 months) is outweighed by the benefits of this approach in avoiding major spine surgery and general anesthesia. In summary, a transforaminal lumbar approach for intrathecal injection represents a safe and effective alternative to surgical procedures for access to the intrathecal space in adult patients with SMA and a history of complicated spine surgery requiring nusinersen treatment.

Metastatic Medullary Carcinoma of Thyroid Presenting as a Dural-Based Mass: Case Report and Review of Literature

Dural metastasis from medullary thyroid carcinoma (MTC) is not well established in English literature. We present the case report of MTC with unusual clinical presentation as a dural-based mass in a 39-year-old male with no family history of multiple endocrine neoplasia syndrome. Magnetic resonance imaging showed an extra-axial dural-based mass in right frontal lobe with calvarium and soft tissue extension to the right superior orbit. Histopathology showed MTC with variegated morphology and various patterns. Thyroid mass and widespread metastases from medullary thyroid carcinoma were subsequently identified.