Alfonso Cerase

Thalidomide-induced neuropathy: A ganglionopathy?

Thalidomide is an “old” drug with new indications in many immune-related and neoplastic diseases. In addition to teratogenicity, peripheral neurotoxicity is the main adverse effect limiting its therapeutic use. The incidence of thalidomide-induced neuropathy is variable, ranging from 1% in leprosy to 70% in prurigo nodularis.1 Clinical features include sensory axonal polyneuropathy with early distal tingling followed by impairment of all modalities of sensation, which may remit after immediate drug withdrawal. Mild motor involvement and severe proprioceptive sensory failure have been reported in severe cases. Prospective neurophysiological studies performed every 3 to 6 months, or after a cumulative dose of 10 g, have shown a sensory nerve action potential (SNAP) amplitude decrease, less than 40% or 50% from baseline, to be the most useful parameter for detecting early nerve damage.1,2⇓ In a 67-year-old man with a 4-year history of biopsy-proven prurigo nodularis unresponsive to steroids and cyclosporine, treatment with thalidomide (100 mg/d) led to complete remission of cutaneous manifestations. Five months after therapy onset, the patient had tingling in hands and feet, followed …

Extramedullary intracranial localization of multiple myeloma and treatment with novel agents: a retrospective survey of 50 patients.

Intracranial involvement in multiple myeloma is extremely rare. The effect of new drugs (eg, thalidomide, bortezomib, lenalidomide) with respect to old drugs (eg, alkylators, steroids) has not been reported.

Two cases of hemichorea-hemiballism with nonketotic hyperglycemia: a new point of view

Hemichorea-hemiballism (HCHB) is an usually continuous, nonpatterned, involuntary movement disorder caused by basal ganglia dysfunction, commonly due to a vascular lesion, described in nonketotic hyperglycemic patients. Particular computed tomography and magnetic resonance imaging findings have been described. The pathogenic mechanism of chorea arising during hyperglycemia and the nature of neuroimaging findings are unclear. In this paper we describe two elderly women with onset of HCHB during a hyperglycemic episode. The symptoms persisted in one of them after recovery of normal glycemia. The pathophysiological mechanism of the disease is discussed in the light of clinical and neuroradiological follow-up.

Hemimegalencephaly in Tuberous Sclerosis Complex

The purpose of this case report is to describe the computed tomographic and magnetic resonance imaging findings of the brain of a 16-month-old girl with an uncommon association between hemimegalencephaly and tuberous sclerosis complex. When a large calcification is found within a hemimegalencephalic cerebral hemisphere, further investigation of a suspected associated tuberous sclerosis complex or another phakomatosis is required to determine pertinent treatment options and genetic counseling. (J Child Neurol 2002;17:677-680).

Endovascular treatment of a cavernous sinus dural arteriovenous fistula by transvenous embolisation through the superior ophthalmic vein via cannulation of a frontal vein

Abstract We describe a new approach for transvenous embolisation of cavernous sinus dural arteriovenous fistulae through the superior ophthalmic vein (SOV), i.e., via percutaneous cannulation of a frontal vein. Modern neurointerventional angiographic materials make it possible to reach the SOV in this way without puncturing it in the orbit or a surgical exposure. Orbital phlebography should still be in the repertoire of interventional neuroradiology units in large centres.

Superselective ophthalmic artery infusion of melphalan for intraocular retinoblastoma: preliminary results from 140 treatments

Purpose:  To report our experience in superselective ophthalmic artery infusion of melphalan (SOAIM) for intraocular retinoblastoma.

Is CT still useful in the study protocol of retinoblastoma

BACKGROUND AND PURPOSE: Intralesional calcium deposition is considered a key element for differentiating retinoblastoma from simulating lesions. Our aim was to assess whether MR imaging associated with ophthalmologic investigations (ophthalmoscopy and ultrasonography) could replace CT in the detection of diagnostic intralesional calcifications in retinoblastoma. MATERIALS AND METHODS: Ophthalmoscopic findings, MR images, CT scans, and histologic examination of 28 retinoblastomas from 23 consecutive children (11 males, 12 females; age range at admission, 1–35 months; mean age, 11 months; median age, 9 months) were retrospectively evaluated. Ultrasonography was performed in 18 patients with 21 retinoblastomas. MR imaging included T2-weighted spin-echo and gradient-echo images, fluid-attenuated inversion recovery images, and T1-weighted spin-echo images with and without contrast enhancement. Clinical data were integrated with MR imaging data to evaluate the utility of both approaches to discover calcifications; particularly, a correlation between intralesional signal-intensity void spots on MR imaging and hyperattenuating areas on CT scans was performed. RESULTS: Ophthalmoscopy detected calcifications in 12 of 28 eyes (42.85%). Ultrasonography detected calcifications in 20 of 21 eyes (95.23%). CT showed hyperattenuating intralesional areas consistent with calcifications in 27 of 28 eyes (96.42%). MR imaging showed intralesional signal-intensity void spots in 25 of 28 eyes (89.28%). All spots detected with MR imaging matched the presence of calcifications on CT scans. Gradient-echo T2*-weighted and fast spin-echo T2-weighted images showed the highest degree of correlation with CT. When we put together ophthalmoscopy, ultrasonography, and MR imaging data, no calcifications detected on CT were missed, and the differential diagnosis was thorough. CONCLUSIONS: A combination of clinical data and MR images may remove potentially harmful ionizing radiation from the study protocol of retinoblastoma.

Uncommon findings in idiopathic hypertrophic cranial pachymeningitis

Abstract.Background:Idiopathic hypertrophic cranial pachymeningitis (IHCP) is a rare, poorly understood, inflammatory disease, usually involving the dura mater of skull base, tentorium, and falx, and presenting with headache, progressive cranial nerve palsies, and cerebellar dysfunction.Patients and Methods:In four patients, the diagnosis of IHCP has been made on the basis of extensive clinical, and radiological investigation, and confirmed by dural biopsy in three patients. The clinical follow-up ranges from 24 to 120 months.Results :At diagnosis, all the patients complained of severe, progressively increasing headache, two had simple or complex partial seizures, but none had cranial nerve palsies. Two patients had electrophysiological evidence of axonal peripheral neuropathy, biopsy-proved in one of them. In all the patients, MRI showed linear or focal thickening of the dura mater of the tentorium and/or of the convexity, sparing the skull base. In one patient, MRI findings resembled chronic subdural hematoma. Dural biopsy demonstrated fibrosis and prominent CD4+ T-cells inflammatory infiltrate. Pachymeningitis was highly responsive to steroid therapy, as was the peripheral neuropathy. In three patients, temporary steroids withdrawal led to dramatic clinical worsening including status epilepticus in one.Conclusions :In the patients here reported, absence of cranial nerve impairment, seizures, MRI findings resembling chronic subdural hematoma, and association with polineuropathy were unusual findings of IHCP. Moreover, the type of inflammatory infiltrate, lacking in previous reported cases, suggests a probable pathogenetic role for cell-mediated immunity of unknown origin.

Intracranial and intraspinal hemorrhage following spinal anesthesia

Spinal anesthesia (SA), accounting for more than 50% of regional anesthesias in the spinal region, is generally perceived as simple and safe. Our purpose is to increase awareness of hemorrhagic complications following SA. A 69-year-old male without either coagulation disorders or anticoagulant/antiplatelet therapy developed acute radiculopathy, and severe mental confusion after SA for prostatectomy. CT showed intracranial subarachnoid and intraventricular acute hemorrhage. Cerebral angiography was negative. MRI showed subarachnoid and subdural hematoma in the dorsolumbar spine. Seven-year follow-up showed permanent cognitive and radicular damage. Multiple attempts for SA most likely caused spinal vessels rupture, either directly or indirectly by inducing differential pressure changes between cerebrospinal fluid and intravascular spaces; however, definite mechanisms have not been completely understood. Patients undergoing spinal puncture must report any neurological abnormality, which may result in irreversible damage. Cases of altered consciousness require an extensive neuroradiological evaluation. Proper competency of physicians responsible for spinal puncture is mandatory.

Cerebral abscesses and necrotic cerebral tumours: differential diagnosis by perfusion-weighted magnetic resonance imaging

PurposeThis study was undertaken to evaluate the usefulness of perfusion-weighted imaging (PWI) in the differential diagnosis of ring-enhancing cerebral lesions, including abscesses, high-grade gliomas and metastases.Materials and methodsNine cerebral abscesses (five pyogenic, four from Toxoplasma gondii), ten glioblastomas and five cerebral metastases in 19 patients were studied with gadolinium-enhanced magnetic resonance imaging, diffusion-weighted imaging (DWI) including calculation of mean apparent diffusion coefficient (ADC) of the lesion core, and PWI. At PWI, the mean of the maximum regional cerebral blood volume (rCBV) was calculated in the gadolinium-enhancing peripheral solid areas and compared with that of the contralateral normal-appearing white matter [ratio=rCBV (lesion)/rCBV (contralateral normal-appearing white matter)].ResultsDWI achieved the differential diagnosis in all cases except for the four Toxoplasma abscesses. At PWI, the mean ratio of the rCBV of the capsular portion was 0.72±0.08 (range 0.60–0.82) in the pyogenic abscesses, 0.84±0.07 (range 0.75–0.91) in the Toxoplasma abscesses, 4.45±1.5 (range 2.9–8.0) in the high-grade gliomas and 3.58±0.68 (range 3.28–4.27) in the metastases.ConclusionsPWI seems to be useful in the differential diagnosis of ring-enhancing cerebral lesions. High rCBV values in the peripheral areas appear to indicate the possibility of a necrotic tumour, whereas low values tend to indicate an abscess.RiassuntoObiettivoValutare l’utilità della perfusione-RM nella diagnosi differenziale delle lesioni cerebrali con “ringenhancement”, quali ascessi, gliomi di alto grado e metastasi.Materiali e metodiNove ascessi cerebrali (5 da piogeni, 4 da Toxoplasma gondii), 10 glioblastomi e 5 metastasi cerebrali in 19 pazienti sono stati studiati con risonanza magnetica con gadolinio endovena, diffusione-RM con calcolo del valore medio del coefficiente di diffusione apparente (DWI-ADC) del centro della lesione e perfusione-RM. Alla perfusione-RM, il valore medio del massimo volume ematico cerebrale regionale (rCBV) è stato calcolato nelle aree solide periferiche e confrontato con quelli della sostanza bianca controlaterale di aspetto normale [ratio=rCBV(lesione)/rCBV(sostanza bianca controlaterale)].RisultatiLa DWI-ADC ha consentito la diagnosi differenziale in tutti i casi, ad eccezione dei 4 ascessi da Toxoplasma gondii. Alla perfusione-RM, la ratio media del rCBV della porzione capsulare è stata di 0,72±0,08 (range: 0,6–0,82) negli ascessi da piogeni, 0,84±0,07 (range: 0,75–0,91) negli ascessi da Toxoplasma gondii, 4,45±1,5 (range: 2,9–8) nei gliomi di alto grado e 3,58±0,68 (range: 3,28–4,27) nelle metastasi.ConclusioniLa perfusione-RM fornisce utili elementi alla diagnosi differenziale delle lesioni cerebrali con “ringenhancement”. Alti valori del rCBV nelle aree periferiche dovrebbero indurre al sospetto di tumore necrotico, mentre bassi valori del rCBV possono orientare verso l’ascesso.