Medullary Lesions Research Articles

Management of pediatric brainstem cavernous malformations: experience over 20 years at the Hospital for Sick Children

Because of their location and biological behavior, brainstem cavernous malformations (CMs) pose a formidable clinical challenge to the neurosurgeon. The optimal management of these lesions requires considerable neurosurgical judgment. Accordingly, the authors reviewed their experience with the management of pediatric brainstem CMs at the Hospital for Sick Children. The authors performed a retrospective chart review of pediatric patients who had received diagnoses of a brainstem CM at the Hospital for Sick Children over the past 20 years. Twenty patients were diagnosed with brainstem CMs. The mean age at diagnosis was 10.1 +/- 5.4 years, and the patients included 13 boys and 7 girls. The mean maximal diameter of the CM was 14.3 +/- 11.2 mm. The lesions were evenly distributed on the right and left sides of the brainstem with 4 midbrain, 13 pontine, and 3 medullary lesions. Seven patients underwent surgery for the management of their CMs, with a mean age at presentation of 5.2 years, and a mean CM size of 21.0 mm. Of note from the surgical group, 2 patients had a family history of CMs, 2 lesions were medullary, the CM reached a pial surface in 6 of 7 patients, and 6 of 7 lesions were located on the right side. The mean age at presentation among the 13 patients in the nonsurgical group was 12.7 years, and the mean CM size was 10.6 mm. Seven of these patients had a prior history of radiation for tumor, and only 3 had lesions that reached a pial surface. The management of brainstem CMs in children is influenced by multiple factors. The majority of patients received conservative management and tended to be asymptomatic with smaller lesions. Patients with larger lesions and direct pial contact, in whom symptoms arose at a younger age were more likely to undergo surgical management. A history of familial CM was also a predictor for receiving surgical treatment. No patients with a prior history of radiation therapy underwent surgery for CMs. The presence of multiple lesions seemed to have no impact on the type of management chosen. Patients who underwent surgery did suffer morbidity related to the procedure, and tended to improve clinically over time. Conservative management was associated with new deficits arising in children, some of which improved with time. Consideration of many clinical and radiological parameters is thus prudent when managing the care of children with brainstem CMs.

Medial Medullary Infarction

Clinical-imaging correlation and long-term clinical outcomes remain to be investigated in medial medullary infarction (MMI). We studied clinical, MRI, and angiographic data of 86 consecutive MMI patients. The lesions were correlated with clinical findings, and long-term outcomes, divided into mild and severe (modified Rankin scale >3), were assessed by telephone interview. Central poststroke pain (CPSP) was defined as persistent pain with visual numeric scale > or = 4. The lesions were located mostly in the rostral medulla (rostral 76%, rostral+middle 16%), while ventro-dorsal lesion patterns include ventral (V, 20%), ventral+middle (VM, 33%), and ventral+middle+dorsal (VMD, 41%). Clinical manifestations included motor dysfunction in 78 patients (91%), sensory dysfunction in 59 (73%), and vertigo/dizziness in 51 (59%), each closely related to involvement of ventral, middle, and dorsal portions, respectively (P<0.001, each). Vertebral artery (VA) atherosclerotic disease relevant to the infarction occurred in 53 (62%) patients, mostly producing atheromatous branch occlusion (ABO). Small vessel disease (SVD) occurred in 24 (28%) patients. ABO was more closely related to VMD (versus V+VM) than was SVD (P=0.035). During follow-up (mean 71 months), 11 patients died, and recurrent strokes occurred in 11. Old age (P=0.001) and severe motor dysfunction at admission (P=0.001) were factors predicting poor prognosis. CPSP, occurring in 21 patients, was closely (P=0.013) related to poor clinical outcome. MMI usually presents with a rostral medullary lesion, with a good clinical ventro-dorsal imaging correlation, caused most frequently by ABO followed by SVD. A significant proportion of patients remain dependent or have CPSP.

Toxoplasmose médullaire au cours de l’infection à VIH

We report the case of an atypical localization of a spinal cord “toxoplasmic abscess”. The 46-year-old patient, HIV-1 positive, was admitted for acute urine retention and gait disorders. MRI revealed a T12–L1 medullary lesion suggesting a tumoral, inflammatory and infectious pathology. The radiological aspect and immunosuppression lead to the initiation of a treatment against Toxoplasma gondii, following the same treatment principles as for cerebral toxoplasmosis. The diagnosis can only be proved by data from autopsy or surgical biopsy, but toxoplasmosis PCR on CSF seems to be an interesting alternative to confirm the diagnosis. According to the literature, PCR is not sensitive enough as a diagnostic tool. Improvement after treatment supported the diagnosis confirmed by PCR.

Sudden Unexpected Death Related to Medullary Brain Lesions

The sudden unexpected death of a person believed healthy has occasionally been followed by a detailed postmortem examination that revealed no cause of death except for the unexpected presence of a medullary brain lesion. Our review of all available cases of sudden unexpected death related to medullary brain lesions (SUD-MBL) revealed the absence of any specific constellation of ante-mortem disease characteristics, together with the finding that major motor and sensory pathways were grossly preserved in most cases. The wide variety in ages of the victims, and in specific types of tissue pathology affecting the medulla, makes this illness extremely difficult to anticipate when the medullary lesions are not otherwise known to exist during life. SUD-MBL may be a specific clinico-neuropathologic disease entity, having significant importance for forensic investigators trying to establish the cause of sudden unexpected death in a victim of any age. Because victims often harbor their medullary lesions for days or weeks before SUD-MBL, clinical physicians as well need to consider the possibility of medullary brain involvement by any disease process, neurologic or systemic, while managing their patients.

Orexin-A Hyperphagia: Hindbrain Participation in Consummatory Feeding Responses

Orexin-A (ORXA) is an orexigenic neuropeptide produced by the lateral hypothalamus that increases food intake when injected into the brain ventricles or forebrain nuclei. We used a licking microstructure analysis to evaluate hindbrain and forebrain ORXA effects in intact and hindbrain-lesioned rats, to identify the motivational and anatomical bases of ORXA hyperphagia. Intact rats with cannulas in the fourth brain ventricle (4V) received vehicle (artificial cerebrospinal fluid) or ORXA (0.1, 0.4, 1, or 10 nm) injections before 90 min access to 0.1 m sucrose. Meal size and frequency were increased in a double-dissociated manner by the 1 and 10 nm doses, respectively. In experiment 2, 4V 1 nm ORXA was applied to rats offered solutions varied in caloric and gustatory intensity (water and 0.1 and 1 m sucrose). ORXA increased meal frequency for all tastants. ORXA increased meal size only for 0.1 m sucrose, by prolonging the meal without affecting early ingestion rate or lick burst size, suggesting that 4V ORXA influenced inhibitory postingestive feedback rather than taste evaluation. In experiment 3, rats with cannulas in the third ventricle (3V) received dorsal medullary lesions centered on the area postrema (APX group) or sham procedures, and licking for water and 0.1 and 1 m sucrose was evaluated after 1 nm 3V ORXA/artificial cerebrospinal fluid injections. The 3V ORXA increased 0.1 m sucrose meal size and meal frequency for all tastants in the sham group, as observed after 4V ORXA in experiment 2. In the APX group, 3V ORXA injections influenced meal frequency, but they no longer increased meal size. However, the APX rats increased meal size for 0.1 m sucrose after food and water deprivation and after 3V angiotensin II injection. They also showed meal size suppression after 3V injection of the melanocortin-3/4 receptor agonist melanotan II (1 nm). These findings suggest that the area postrema and subjacent nucleus of the solitary tract are necessary for increases in consummatory (meal size) but not appetitive (meal frequency) responses to 3V ORXA. The meal size increases may be due to reduced postingestive feedback inhibition induced by ORXA delivered to either the hindbrain or forebrain ventricles.

Impaired Opening of the Upper Esophageal Sphincter in Patients with Medullary Infarctions

The aim of this study was to report on nine dysphagic patients with medullary infarction and to evaluate swallowing characteristics based on the location of the lesions.We retrospectively reviewed the medical records of these nine patients. The medullary lesions were midlateral (three patients), dorsolateral (one patient), inferodorsolateral (four patients), and paramedian (one patient). The levels of the lesions were upper (four patients), middle (two patients), upper and middle (two patients), and middle and lower medulla (one patient). Dysphagia after medullary infarction was more common in patients with upper or middle medullary level and dorsolateral medullary level lesions. The common findings on videofluoroscopic swallowing studies in patients with lateral medullary infarctions were impaired upper esophageal sphincter opening, aspiration from pyriform sinuses' residue caused by pharyngeal weakness, and multiple swallowing to clear boluses from the pharynx to the esophagus. In patients with medullary infarctions, the lesion levels and loci and their related clinical findings can be useful in predicting dysphagia and aspiration. Because severe dysphagia with serious complication is very common in patients with medullary infarctions, active diagnostic and therapeutic approaches are needed.

Transient Urinary Retention in Acute Right Lateral Medullary Infarction

Urinary disturbance has rarely been reported in patients with a medullary lesion. We evaluated a patient with acute infarction in the right lateral medulla who had voiding difficulty as an initial manifestation. Urodynamic study showed detrusor areflexia on voiding, and the voiding symptom completely recovered 2 weeks after the stroke onset. The urinary retention in this patient might be caused by interruption of descending fibers from facilitatory pontine tegmentum micturition centers.

Modificação técnica para correção de coarctação aórtica com hipoplasia do arco aórtico

To study technical feasibility and early results of the technical modification suggested by Caliani et al. for correction of aortic coarctation and aortic arc. Between January 2005 and July 2006, nine newborn patients with aortic coarctation and significant aortic hypoplasia were selected, and underwent a new surgical approach in order to correct this aortic defect. The definition of aortic arch hypoplasia according to Moulaert's criteria is an aortic arch with a diameter that is less than 50% of the diameter of the ascending aorta. In this study, only patients with proximal and distal aortic hypoplasia were selected. Many techniques were previously used, but significant residual gradients were observed, as well as the incovenience of definitive occlusion of the left subclavian artery. The aim of this study is to describe a new surgical technique that includes left posterolateral thoracotomy, wide mobilization of descending aorta with occlusion of the first two intercostal branches, transection of the left subclavian artery at its base, wide resection of the hypoplastic area and the surrounding regions of the ductus arteriosus; end-to-end anastomosis between the aortic arch and descending aorta, with 7-0 PDS thread and reimplantation of the subclavian artery into the left carotid artery with side-to-end anastomosis. There were no perioperative or late deaths. The mean residual gradient was 5 mmHg. Up to now, there were no cases of recoarctation or medullary neurological lesions. Despite the small number of cases and the short follow-up, this technique modification may be an excellent option for the treatment of this complex situation.

Peripheral Type Facial Palsy in a Patient with Dorsolateral Medullary Infarction with Infranuclear Involvement of the Caudal Pons

The corticobulbar tract fibers descend near the corticospinal tract, mostly to the upper medulla, where they decussate and ascend in the dorsolateral medulla to connect with the contralateral facial nucleus. Therefore, central type facial palsy can be present in patients with ipsilateral dorsolateral upper medullar lesion. We describe a 71-year-old man with lateral medullary infarction who showed ipsilateral peripheral type facial palsy. Brain diffusion-weighted image showed hyperintensities on the left dorsolateral portion of upper medulla and adjacent inferomedial tegmentum of the lower pons. Transfemoral cerebral angiography depicted prominence of ipsilateral posterior inferior cerebellar artery with focal stenosis. Left posterior inferior cerebellar artery might supply the inferolateral tegmentum of the lower pons, which is usually supplied from anterior inferior cerebellar artery. The peripheral type facial palsy in our patient may have resulted from facial infranuclear involvement of the caudal pons extended from dorsolateral upper medullary lesion in ascending pathway of corticobulbar tract fibers.

Orthostatic hypotension associated with dorsal medullary cavernous angioma

Orthostatic hypotension (OH) is a rare manifestation of medulla oblongata lesions that may be because of interruption of descending sympathoexcitatory axons. To illustrate the location of a medullary lesion that produced OH following resection in relationship to the location of putative sympathoexcitatory pathways. A case with dorsal medullary cavernous angioma presenting with OH is described. The possible localization of lesion was compared with distribution of tyrosine hydroxylase (TH)-immunoreactive axons in a comparable section of the medulla of a control brain. The patient had marked OH after partial removal of the cavernous angioma. Biopsy confirmed the diagnosis. The magnetic resonance imaging location of the lesion overlapped that of TH-immunoreactive axons of the medullary transtegmental tract. A restricted lesion of medullary lesion interrupting the catecholaminergic transtegmental tract arising from the sympathoexcitatory C1 neurons of the rostral ventrolateral medulla could result in severe OH.

The importance of combined hematologic and CT diagnosis for monitoring response in patients with multiple myeloma treated with Bortezomib based therapy

8597 Background: To assess the potential role of radiological monitoring (conventional CT) of lytic bone lesions for diagnosis of progressive (PD) or relapsing (R) multiple myeloma (MM) in comparison with laboratory-based diagnosis, soft-tissue based CT-diagnosis and combined hematologic-radiological diagnosis. Materials and Methods: Between 3/03 and 12/07, 201 patients with MM underwent nonenhanced whole-body low-dose multidetector-CT (WBLD-MDCT) survey parallel to hematological follow up (582 investigations). CT-scans were performed using a standardized low-dose protocol assessing number and size of lytic bone lesions (bone window setting, bW) similar to conventional X-ray diagnosis. In addition, number, size and density of medullary and extra-medullary lesions (soft-tissue window setting, s-tW) were analyzed. Progressive or relapsing myeloma was defined by progression of lytic bone lesions, medullary and extra-medullary tumor infiltration and/or of established basic hematologic parameters. Results: 210/582 investigations showed PD or R. 50/210 (24%) yielded the correct diagnosis by all three methods, 50/210 (24%) were diagnosed correctly by WBLD-MDCT (bW), 133/210 (63%) by laboratory-based testing and 195/210 (93%) by WBLD-MDCT (s-tW) as well as by the combination of the latter two. 8/26 assessments of R were biomarker negative. Only 1 of these examinations proved positive on WBLD-MDCT (bW) while the other 7 were correctly diagnosed by WBLD-MDCT (s-tW) due to progress of medullary infiltration and/or extra-medullary manifestation. Of the biomarker-negative assessments of PD (84/210), only 24 (24/84) showed progress of osteolysis on WBLD-MDCT (bW) while all were correctly assessed by WBLD-MDCT (s-tW). Discussion: This retrospective analysis indicates that monitoring of lytic bone lesions in patients with MM is inaccurate for assessment of progression or relapse of multiple myeloma. Due to the effects of specific chemotherapy and accompanying bisphosphonates therapy, progression of lytic bone lesions seems to occur much later in the course of the disease showing slower growth kinetics compared with medullary and extra-medullary myeloma manifestations. No significant financial relationships to disclose.

Lesions of medullary catecholaminergic neurons increase salt intake in rats

Several findings suggest that catecholaminergic neurons in the caudal ventrolateral medulla (CVLM) contribute to body fluid homeostasis and cardiovascular regulation. From the CVLM other areas in central nervous system involved in cardiovascular regulation and hydroelectrolyte balance can be activated. Therefore, the aim of the present study was to investigate the effects of lesions of these neurons on 0.3 M NaCl and water intake induced by subcutaneous injection of furosemide (FURO) + captopril (CAP) or 36 h of water deprivation/partial hydration with only water (WD/PR). Male Wistar rats (320–360 g) were submitted to medullary catecholaminergic neuron lesions by microinjection of anti-dopamine-β-hydroxylase-saporin (anti-DβH-saporin; 6.3 ng in 60 nl) into the CVLM (SAP-rats). Sham rats received microinjections of free saporin (1.3 ng in 60 nl) in the same region. In SAP-rats, the 0.3 M NaCl intake was increased after FURO + CAP (6.8 ± 1.0 ml/2 h, vs. sham: 3.7 ± 0.7 ml/2 h) as well as after WD/PR (11.1 ± 1.3 ml/2 h vs. sham: 6.1 ± 1.8 ml/2 h). Conversely, in SAP-rats, the water intake induced by FURO + CAP (14.8 ± 1.3 ml/2 h, vs. sham: 14.1 ± 1.6 ml/2 h) or by WD/PR (3.6 ± 0.9 ml/2 h, vs. sham: 3.2 ± 1.1 ml/2 h) was not different from sham rats. Immunohistochemical analysis indicates that microinjections of anti-DβH-saporin produced extensive destruction within the A1 cell groups in the CVLM. These results suggest an inhibitory role for medullary catecholaminergic neurons on sodium appetite.

Hyperattenuating bone marrow abnormalities in myeloma patients using whole-body non-enhanced low-dose MDCT: correlation with haematological parameters

We aimed to establish the role of hyperattenuating medullary abnormalities detected by whole-body non-enhanced low-dose multidetector CT (WBLD-MDCT) in multiple myeloma (MM) patients referred for primary evaluation. 50 consecutive patients with untreated Stage I (n(I) = 11), Stage II (n(II) = 10) and Stage III (n(III) = 29) MM underwent WBLD-MDCT for staging. The number and size of osteolysis, as well as haematologic parameters including paraprotein and beta2-microglobulin levels, were assessed and related to the number, size and density of medullary abnormalities assumed to represent myeloma involvement. Bone marrow abnormalities were found in 2/11 (18%) Stage I, 6/10 (60%) Stage II and 20/29 (69%) Stage III myeloma patients, and did not parallel the incidence of osteolysis. Patients with medullary lesions had higher levels of immunoglobulin A (median, 4730 mg dl(-1) vs 1520 mg dl(-1)), light-chain proteinuria (median, 690 mg dl(-1) vs 214 mg dl(-1)) and IgG paraprotein (median, 3270 mg dl(-1) vs 2610 mg dl(-1)) compared with patients without medullary lesions. In patients with medullary abnormalities, levels of serum beta2-microglobulin were significantly higher than in patients without detectable marrow infiltrates (median, 4.3 mg dl(-1) vs 2.4 mg dl(-1); p = 0.0015). In conclusion, medullary abnormalities visualized by WBLD-MDCT are encountered in all stages of myeloma, including cases without osteolysis. They are associated with significantly elevated serum levels of paraprotein (reflecting tumour mass) and beta2-microglobulin, a prospective prognostic marker for myeloma. The nature and possible prognostic significance of medullary abnormalities detected by WBLD-MDCT therefore warrants further investigation.

Saccadic adaptation in lateral medullary and cerebellar infarction

To determine the adaptive capability of saccadic eye movements, and its association with enduring saccadic dysmetria in cerebellar and lateral medullary infarction (LMI), we investigated saccadic accuracy and adaptation in 15 patients with cerebellar or lateral medullary infarction, compared with those of 7 patients with diffuse cerebellar atrophy and 11 normal subjects. Saccade adaptation was elicited by a 37.5% backward target step during the primary saccade in both horizontal directions. Horizontal preadaptive saccadic gains were decreased in patients with cerebellar infarction, and contralesionally in patients with LMI. In contrast, adaptive saccadic gain change was reduced in patients with diffuse cerebellar atrophy and cerebellar infarction. Saccadic hypometria and reduced saccadic adaptability were dissociated in the majority of the patients with cerebellar infarctions; seven of the eight patients with cerebellar infarction showed saccadic hypometria and only three of them showed reduced saccadic adaptation, uni- or bilaterally in two with bilateral infarctions and ipsilesionally in one with unilateral infarction. The most commonly affected structure on MRI was the cerebellar hemisphere in the patients either with saccadic hypometria or with reduced saccadic adaptation. All patients with unilateral LMI exhibited normal saccadic gain adaptation in both directions, including those patients with enduring saccadic ipsipulsion. Our results suggest that the cerebellar hemispheres as well as the dorsal vermis and fastigial nucleus may be involved in the control of saccadic accuracy and adaptation. Reduced saccadic adaptation and persisting dysmetria are not tightly linked to each other in the cerebellar or lateral medullary lesions.

Unusual Radiologic Presentation of Monostotic Fibrous Dysplasia

Fibrous dysplasia is a benign fibro-osseous lesion that can occur as an isolated skeletal lesion (monostotic form) or affect multiple skeletal sites (polyostotic form). In addition, fibrous dysplasia may be associated with single or multiple endocrinopathies, or with precocious puberty and cutaneous hyperpigmentation in McCune-Albright syndrome. Fibrous dysplasia usually appears as a well-defined radiolucent medullary lesion that is irregular, mildly expansive, and with a hazy opacity classically described as "ground glass" in appearance. In the long tubular bones, fibrous dysplasia may cause expansion of the bone contour with cortical thinning and endosteal scalloping. The shaft is typically involved, but the metaphysis may also be affected. These changes are usually recognizable on plain radiographs, but in cases where the lesion is difficult to visualize computed tomography may be beneficial. However, fibrous dysplasia may present with radiographic features that may mimic other benign fibro-osseous lesions, may be associated with other lesions, and even be confused with certain types of malignancies. We present a case of monostotic fibrous dysplasia in the proximal tibia of an 11-year-old girl with the unusual radiologic features of multifocal lytic lesions and cortical disruption. While most often the clinical presentation, laboratory findings, and imaging studies permit the diagnosis of fibrous dysplasia, in certain cases this may pose a diagnostic challenge. The diagnosis was confirmed by open biopsy.

Pathology and immunopathology of immunoglobulin G4‐related sclerosing cholangitis: The latest addition to the sclerosing cholangitis family

Sclerosing cholangitis is heterogeneous in its etiopathogenesis. Recently, sclerosing cholangitis showing abundant immunoglobulin (Ig)G4+ plasma cell infiltration was added to the sclerosing cholangitis group. This form was frequently associated with sclerosing pancreatitis (autoimmune pancreatitis) and also occasionally with other diseases such as chronic sclerosing sialadenitis, all of which falls within IgG4-related sclerosing disease. Herein, this new member, called IgG4-related sclerosing cholangitis (IgG4-SC), is reviewed. IgG4-SC shows grossly medullary and fleshy lesions along the biliary tree, and histologically marked lymphoplasmacytic infiltration with extensive fibrosis, and obliterative phlebitis, sharing histopathological features with sclerosing pancreatitis. Peribiliary glands are also severely affected. Interestingly, hepatic inflammatory pseudotumor (HIP) is not infrequently associated with IgG4-SC, and is thought as a local exaggeration of IgG4-SC. Immunohistochemically, many IgG4+ plasma cells and CD4+/CD25+ regulatory T cells are found around the affected bile ducts and portal tracts. Incontrast, these cells are scarce in the affected bile ducts of primary sclerosing cholangitis (PSC), a prototype of sclerosing cholangitis. Biliary lining epithelia are relatively spared in IgG4-SC in comparison with those of PSC showing degeneration and ulceration. In some cases of IgG4-SC, IgG4+ plasma cells are also found considerably in small portal tracts, so needle liver biopsy is useful for the diagnosis of IgG4-SC. Therapeutically, IgG4-SC responds well to steroid therapy, while such character is not reported in PSC. Taken together, IgG4-SC may be etiologically different from PSC, and immunopathological processes relating to IgG4 and regulatory T cells may be involved in the pathogenesis of IgG4-SC. Further studies are needed to clarify the etiopathogenesis of IgG4-SC and its related disorders.

Medullary infarcts may cause ipsilateral masseter reflex abnormalities

There is a suprasegmental influence on the masseter reflex (MassR) in animals, which is mediated via the fifth nerve spinal nucleus (5SpN). Corresponding data in humans are lacking. Out of 268 prospectively recruited patients with clinical signs of acute brainstem infarctions, we identified 38 with magnetic resonance imaging (MRI)-documented unilateral infarcts caudal to the levels of the fifth nerve motor and main sensory nuclei. All had biplanar T2- and echo planar diffusion-weighted MRI and MassR testing. Five patients (13%) had ipsilateral MassR abnormalities. In all, the infarcts involved the region of the 5SpN. Patients with medullary infarcts involving the region of the 5SpN may thus have ipsilateral MassR abnormalities. This possibly represents an interruption of an excitatory projection mediated via the 5SpN to masseter motoneurons in the fifth nerve motor nucleus. MassR abnormalities with medullary lesions restrict the topodiagnostic value of the MassR.

Neuro-Behçet: MR study of a group of patients

Behçet's disease is a chronic inflammatory, multisystemic disease of unknown aetiology. Central nervous system involvement occurs in a variable proportion of cases (4 to 49%) and is due to intracranial hypertension secondary to dural sinus thrombosis, inflammatory parenquimal lesions or recurrent meningoencephalitis. We reviewed 12 patients, 7 men and 5 women, aged between 27 to 40 years at the time of diagnosis. Neurological manifestations were secondary to parenquimal lesions in 7 patients, meningoencephalitis in 3 patients (including one with extensive medullary lesion) and dural sinus thrombosis in 2. Magnetic Resonance (MR) findings in Neuro-Behçet are non-specific. Nevertheless, MR has a role in characterizing brain lesions topography, helping in the diagnosis and in the follow-up of these patients.

Paraneoplastic vasculitis of central nervous system presenting as recurrent cryptogenic stroke

We report the case of paraneoplastic vasculitis of the central nervous system associated with breast cancer. A 54-year old woman had a complete workup because of recidive seizures. Magnetic resonance imaging (MRI) showed cerebral and medullary cryptogenic lesions, and a diagnosis of encephalomyelitis of unknown origin was made. She was treated with corticosteroids, but because of cognitive impairment and recurrence of epileptic seizures, she was finally transferred to our Neurology Department. The previous diagnosis was then changed, because a poorly differentiated invasive lobular breast cancer was discovered. Thoracic and abdominal computed tomography (CT) scans diagnosed pulmonary, pericardial, adrenal, and renal metastases. Because MRI findings did not fit with the diagnosis of brain metastases, a CT-guided cerebral biopsy was performed, and she was eventually diagnosed with paraneoplastic vasculitis of the central nervous system.

Head-shaking nystagmus and new technology

The clinical specialty of otoneurology has recently undergone a major change due to the availability of new instruments based on small video cameras. Eye movements and nystagmus can be observed at the bedside with extremely high sensitivity (and relatively low cost), using “video-Frenzel goggle” devices. These resemble scuba goggles having cameras set over the eyes, and infrared illumination which allows observation of the eyes in total darkness. As the image of the eyeball is typically displayed on a large screen TV, and total darkness is easy to obtain, small details of nystagmus can easily be appreciated. This gigantic improvement in methodology has begun to bear fruit with refinements of the bedside examination. An example is the article by Choi et al. in this issue,1 which is concerned with head-shaking nystagmus (HSN) in persons with medullary lesions. HSN, described in 1907 by Barany,2 is a provoked jerk nystagmus …