Diagnostic pathway to amyotrophic lateral sclerosis (ALS): the significance of Babinski's sign in a unique patient and the necessity for a multidisciplinary approach.

In this case report, we present a patient with rare diagnosis of amyotrophic lateral sclerosis (ALS). In this patient, four different neurological disorders were diagnosed in a short-time period of 8 months. The first three diagnoses, chronic low back and leg pain, a left frontal gemistocytic astrocytoma WHO grade 2 and suspected Lyme's disease, could not fully explain the signs of physical examination. Finally, the diagnosis of ALS could reduce these signs to the same denominator.

Magnetic resonance T2 image signal intensity ratio and clinical manifestation predict prognosis after surgical intervention for cervical spondylotic myelopathy.

STUDY DESIGN: A retrospective imaging study of 73 patients who underwent surgery for cervical spondylotic myelopathy (CSM) between April 2005 and July 2007. OBJECTIVE: To investigate whether magnetic resonance (MR) T2 image signal intensity (SI) ratio and clinical manifestation can assess the prognosis in patients with CSM. SUMMARY OF BACKGROUND DATA: The association between intramedullary high SI on T2-weighted MR images and surgical outcome in CSM remains controversial. The means of quantizing SI ratio for the disease has not been discussed. METHODS: A total of 73 patients with cervical compressive myelopathy were retrospectively enrolled and were treated with anterior, posterior, and posterior-anterior united decompression. A total of 1.5-T magnetic resonance imaging was performed in all patients before surgery. T2-weighted images of sagittal increased SI on the cervical spinal cord were obtained, and the regions of interest (ROIs) are taken by 0.05 cm. T2-weighted MR images of sagittal normal cord SI on the cervical between C7-T1 disc levels were obtained, and the ROIs are taken by 0.3 cm. SI value is measured by computer, and the SI ratio between the regions 0.05 cm and 0.3 cm has been calculated. If no intramedullary high SI was noted on T2-weighted MR images, the ROIs were taken by 0.05 cm of the severe compression cord. All patients had been divided into 3 groups by hierarchical clustering analysis with SI ratio (Group 1: low SI ratio, Group 2: middle SI ratio, and Group 3: high SI ratio). Statistical analyses were performed with SPSS 11.0. RESULTS: There are significant differences between 3 groups by comparing the recovery rate (P < 0.001), age (P = 0.003), duration of disease (P = 0.001), Babinski sign (P < 0.001), preoperative JOA score (P = 0.006), and postoperative JOA score (P < 0.001). There are no significant differences on sex among 3 groups (P = 0.387). By using the multiple comparison analysis, the above results are further shown. CONCLUSION: Patients with low SI ratio who were not too old and had a shorter duration of disease experienced a good surgical outcome. However, with the increase of SI ratio and the occurrence of pyramidal sign, a poor prognosis after surgery will show. SI ratio and clinical manifestation can be a predictor of surgical outcome.

Posterior reversible encephalopathy syndrome: long-term follow-up.

BACKGROUND: Posterior reversible encephalopathy syndrome (PRES) has been known for more than 10 years. The long-term prognosis of this condition remains unknown. PATIENTS AND METHODS: In 2006, the authors screened retrospectively the medical records of our department between 1993 and 2006 for PRES. The authors identified 13 patients. Since 2006, another 12 patients have been included prospectively. Since then, follow-up has been performed yearly for all patients. They were investigated in the outpatient clinic or, if they declined to attend, were interviewed by telephone. RESULTS: The authors identified 25 patients with 27 episodes of PRES. Eighty-four per cent of the patients had generalised seizures. Their mean blood pressure was 167/100 mm Hg. Follow-up was performed for all patients over a mean period of 2250 days (range 59-9396; median 1699). Symptoms resolved, on average, after 7.5 days. Restitution of imaging abnormalities could be shown in 72% of cases. All others showed a clear improvement, but without complete restitution, after a mean duration of 41 days. Recurrence of PRES was observed in two patients (8%), 3 years after complete recovery from their first episode. CONCLUSION: These data show that PRES has a good short-term and long-term prognosis. Recurrence is infrequent, even though trigger factors for PRES were repeatedly experienced by the patients. Resolution of MRI lesions is slower than clinical recovery.

Two Babinski signs in seropositive (HAM) and seronegative tropical spastic paraparesis.

Tropical spastic paraparesis (TSP) may or may not be associated to HTLV-I antibodies and is usually characterized by clinical and pathological spinal cord abnormalities at thoracic levels. We present here five Brazilian patients who had typical chronic idiopathic spastic paraparesis; two of them were HTLV-I seropositive (HAM) and three HTLV-I seronegative (TSP) -associated-myelopathy. Three out of these five patients also displayed clinical supraspinal involvement, indeed, platysma muscle hypotrophy or atrophy (the Babinski plus sign). These findings support the view that clinical involvement in HAM and TSP is wider than the spinal cord abnormalities usually considered. Possible non-infectious co-factors (e.g., mycotoxins) may be involved in disease pathogenesis in a multistep process of viruses, toxins and environment which may account for serological differences found in this group of patients.

Two Babinski signs in seropositive (HAM) and seronegative tropical spastic paraparesis / Dos signos de Babinski en pacientes con paraparesia espástica tropical seropositiva (HAM) y seronegativa

Tropical spastic paraparesis (TSP) may or may not be associated to HTLV-I antibodies and is usually characterized by clinical and pathological spinal cord abnormalities at thoracic levels. We present here five Brazilian patients who had typical chronic idiopatic spastic paraparesis; two of them were HTLV-I seropositive (HAM) and three HTLV-I seronegative (TSP) - associated-myelopathy. Three out of these five patients also displayed clinical supraspinal involvement, indeed, platysma muscle hypotrophy or atrophy (the Babinski plus sign). These findings support the view that clinical involvement in HAM and TSP is wider than the spinal cord abnormalities usually considered. Possible non-infectious co-factors (e.g., mycotoxins) may be involved in disease pathogenesis in a multistep process of viruses, toxins and environment which may account for serological differences found in this group of patients.

La paraparesia espástica tropical (PET), puede o no estar asociada con anticuerpos contra el HTLV-I y se caracteriza, usualmente, por alteraciones clínicas y patológicas a nivel de region dorso-lumbar de la medula espinal. Presentamos cinco pacientes brasileros, quienes tuvieron hallazgos típicos de paraparesia espástica crónica idiopática; dos de ellos tuvieron (HAM) y tres no tuvieron (TSP) anticuerpos, en el suero, contra el HTLV-I. En tres pacientes se encontró hipotrofia o atrofia del músculo platisma (signo de Babinski plus), demostrando que el compromiso clínico en pacientes con HAM y TSP se extiende más allá de la médula espinal torácica. Cofactores (por ejemplo, micotoxinas) podrían estar involucrados en la patogénesis de esta enfermedad, en una interacción compleja de virus, toxinas y medio ambiente, lo cual explicaría las diferencias serológicas encontradas en este grupo de pacientes.

[Brugada syndrome and other cardiovascular abnormalities related to tricyclic antidepressants ans related drugs intoxication]. / Syndrome de Brugada et autres anomalies cardiovasculaires associées à l'intoxication par les antidépresseurs tricycliques et apparentés.

UNLABELLED: The aim of this paper is to report major cardiovascular complications related to intoxication due to tricyclic antidepressants (TCA) and related drugs, especially the stabilizing membrane effect (SME) and Brugada syndrome, and identify risk factors related to cardiac toxicity. POPULATION AND METHODS: A retrospective study (35 months), including all adult patients admitted for an isolated intoxication by a TCA or a related drug. The statistical analysis of clinical signs and history as well as ECG abnormalities included parameters recorded in emergency rooms. RESULTS: 65 patients without underlying cardiovascular history were retrieved (mean age 30 + 12 years). The intoxication was intentional in all cases, and the mean estimated taken dosage (ETD) was at 749 + 436 mg. Amitriptyline was the most common drug (66%) followed by clomipramine (29%). The cardiovascular examination noted a tachycardia in 63% of cases (mean HR - 108 + 13 bpm), followed by hypotension (SBP = 80 + 40 mmHg) in 11% of cases. The ECG showed a sinus tachycardia (63%), a PR prolongation (>200 ms) in 28% of cases, a MSE (15.4%) and a type-I aspect of Brugada syndrome (15.4%). The analysis of influence of ETD on HR showed a linear relationship, with the equation HR = 82.276 + 0.039 EDT (mg) (R2=0.138, p=0.001). Analyzing the influence of type of drug on ECG abnormalities revealed a more tachycardia effect of amitryptiline vs. clomipramine (p=0.047). CONCLUSION: Tachycardia is a frequent sign of intoxication, which can be expressed by a linear function depending on ETD. The MSE is more frequently noted with amitriptyline. The clinical aspect of type-I Brugada syndrome is as frequent as the MSE but does not seem prognostic. It is unclear whether it is related to an isolated electrical aspect or it hides a genetic mutation.

Acute spinal cord compression in hereditary multiple exostoses.

Osteocartilaginous exostoses are benign bone tumors frequently found in the metaphysis of long bones but rarely in the spine. Four patients with acute spinal cord decompensation due to vertebral exostoses spinal cord compression have been previously described in the literature. We report an additional case of rapidly evolving spinal cord compression due to a cervical osteochondroma in a patient with hereditary multiple exostoses (HME), also known as Bessel Hagen disease. Careful analysis of the 5 cases suggested to us that patients with HME should have a systematic spinal imaging screening, in order to prevent rapid neurological decompensation. A minimal risk surgical procedure can be performed at a time of election.

Constant and severe involvement of Betz cells in corticobasal degeneration is not consistent with pyramidal signs: a clinicopathological study of ten autopsy cases.

This report concerns a clinicopathological study of three additional patients with corticobasal degeneration (CBD), described here for the first time, and a clinicopathological correlation between pyramidal signs and upper motor neuron involvement, in ten autopsy cases of CBD, including seven cases reported by us previously. We investigated pyramidal signs, including hyperreflexia, Babinski sign, and spasticity, and involvement of the primary motor cortex and pyramidal tract, focusing on the astrocytosis of the fifth layer of the primary motor cortex. Pyramidal signs were observed in six (60%) of the ten cases. Hyperreflexia was evident in six patients (60%), with spasticity being observed in three patients (30%). Loss of Betz cells associated with prominent astrocytosis and presence of ballooned neurons in the fifth layer of the primary motor cortex was observed in all ten cases. In all cases, involvement of the pyramidal tract was obvious in the medulla oblongata, without involvement of the pyramidal tract in the midbrain. Constant and severe involvement of the fifth layer of the primary motor cortex, including the Betz cells, has not previously been reported in CBD. We suggest that the pyramidal signs in CBD have been disregarded.

An immunohistochemical study of extracellular matrix proteins laminin, fibronectin and type IV collagen in paediatric glioblastoma multiforme.

AIMS: In the recent decades many studies have been addressed in the literature to assess specific factors related to glioblastoma multiforme (GBM) invasion. However, few studies have evaluated tumour cell's interaction with specific extracellular matrix (ECM) components, and, moreover, there is a lack of information regarding the occurrence of these phenomena in paediatric GBM. METHODS AND RESULTS: ECM proteins were evaluated in six cases of paediatric GBM assessing the immunohistochemical expression of laminin, fibronectin, and type IV collagen. We used a semiquantitative scale, ranging from not detected (zero) to marked (3). Laminin expression was minimal in three cases, moderate in one case, marked and generalised in one patient and marked and focal in the last case. Fibronectin expression was minimal in three patients; moderate immunoreactivity was documented in one case. Conversely, one case was classified as marked with generalised distribution and the remaining case as marked with focal immunostaining. Type IV collagen expression was minimal in three cases, moderate in one, marked with focal reaction in one and marked with generalised reaction in the remaining case. CONCLUSIONS: This study provides additional insights into tumour invasion features of paediatric GBM, as ECM plays a pivotal role in numerous cellular functions during normal and pathological processes. Although based on a limited number of patients, this investigation may serve as a challenge for the management of paediatric GBM, stimulating trials with larger patient numbers aimed at documenting specific factors influencing GBM prognosis.

[Diagnostic value of electromyography in revealing the laterality of the motor deficit in brain tumors]. / Diagnosticheskaia tsennost' élektromiografii dlia vyiavleniia storonnosti motornogo defitsita pri opukholevykh zabolevaniiakh golovnogo mozga.

Electromyographic studies of anterior tibial muscles supplemented by simultaneous induction of Babinski's reflex indicated the appearance of bioelectrical activity in the extremities contralateral to the tumour which suggested the generalization of tone alterations and helped determine the laterization of the pathological process even when there were no clinical manifestations of motor deficit. Having analyzed their own and literature data, the authors came to the conclusion that the contralateral response observed on the electromyogram in the form of prolonged bioelectrical activity with simultaneous appearance of tonic tension in the crus flexors in the absence of clinically evident motor deficit is explained by the presence of the crossover protective reflex on the spinal level.

Persistent neurological deficit precipitated by hot bath test in multiple sclerosis.

For a half century, the hot bath test has been used as a "diagnostic test" in multiple sclerosis. The appearance of new neurological signs or aggravation of preexisting signs generally is transient, with resolution on return of body temperature to normal. We have observed four patients, however, with considerable and prolonged neurological debilitation after hot bath testing. We suggest caution in the application of such testing.