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Klebsiella pneumoniae liver abscess associated with septic spinal epidural abscess.

A 56-year-old Japanese man with hypertension presented with a 10 days history of high fever, right and left upper quadrant tenderness. An abdominal ultrasonography and computerized tomographic scan revealed a large collection in the right lobe of the liver that was consistent with an abscess. A drainage catheter was placed and purulent fluid was drained. Cultures of the fluid and blood were positive for a strain of ampicillin-resistant Klebsiella pneumoniae. Six days after admission, paraplegia and urinary retention were found. On the neurological examination, deep tendon reflexes of the lower extremities were absent bilaterally. Magnetic resonance imaging scan detected thoracic spinal epidural abscess and paraspinal abscess. He received the emergent decompressive laminectomy. Culture of surgical specimen grew ampicillin-resistant K. pneumoniae. The patient was treated with biapenem intravenously. Thereafter, clinical symptoms improved gradually and he was removed to the professional hospital to continue rehabilitation for gait disturbance on hospital day 147.

Journal Article↗

Cauda equina syndrome after epidural steroid injection: a case report.

OBJECTIVE: Conventional treatment methods of lumbusacral radiculopathy are physical therapy, epidural steroid injections, oral medications, and spinal manipulative therapy. Cauda equina syndrome is a rare complication of epidural anesthesia. The following case is a report of cauda equina syndrome possibly caused by epidural injection of triamcinolone and bupivacaine. CLINICAL FEATURES: A 50-year-old woman with low back and right leg pain was scheduled for epidural steroid injection. INTERVENTION AND OUTCOME: An 18-gauge Touhy needle was inserted until loss of resistance occurred at the L4-5 level. Spread of the contrast medium within the epidural space was determined by radiographic imaging. After verifying the epidural space, bupivacaine and triamcinolone diacetate were injected. After the injection, there was a reduction in radicular symptoms. Three hours later, she complained of perineal numbness and lower extremity weakness. The neurologic evaluation revealed loss of sensation in the saddle area and medial aspect of her right leg. There was a decrease in the perception of pinprick test. Deep-tendon reflexes were decreased especially in the right leg. She was unable to urinate. The patient's symptoms improved slightly over the next few hours. She had a gradual return of motor function and ability of feeling Foley catheter. All of the symptoms were completely resolved over the next 8 hours. CONCLUSION: Complications associated with epidural steroid injections are rare. Clinical examination and continued vigilance for neurologic deterioration after epidural steroid injections is important.

Anesthetics, Local↗

Isolated abducens nerve palsy as a regional variant of Guillain-Barré syndrome.

The authors reviewed clinical profiles and laboratory findings for 100 cases of abducens nerve paresis without impairment of the other cranial nerves, limb weakness, and ataxia throughout the clinical course. Review of the medical records of 9300 patients referred to our neuoroimmunological laboratory for serum anti-ganglioside antibody testing. Information was obtained from each primary physician on symptoms of preceding infection; initial symptoms; neurological signs during the illness; the clinical course; treatment provided; and outcome. Isolated abducens nerve paresis was present in 100 patients and bilateral paresis in 29. Tentative diagnoses made by the primary physicians on request of anti-ganglioside antibody testing were abducens nerve palsy (n = 68), Fisher syndrome (n = 14), acute ophthalmoparesis without ataxia (n = 14). Symptoms of infection anteceded in 63. Tendon reflexes were absent or decreased in 27. Distal paresthesias were experienced by seven. Serum anti-GQ1b antibody was positive in 25. These findings suggest that some cases of isolated abducens nerve palsy can be categorized as a regional variant of Guillain-Barré syndrome or mild form of Fisher syndrome.

Abducens Nerve↗

16q-linked autosomal dominant cerebellar ataxia: a clinical and genetic study.

The autosomal dominant cerebellar ataxias (ADCAs) comprise a genetically and clinically heterogenous group of neurodegenerative disorders. Very recently, a C-to-T single nucleotide substitution in the puratrophin-1 gene was found to be strongly associated with a form of ADCA linked to chromosome 16q22.1 (16q-linked ADCA; OMIM 600223). We found the C-to-T substitution in the puratrophin-1 gene in 20 patients with ataxia (16 heterozygotes and four homozygotes) and four asymptomatic carriers in 9 of 24 families with an unknown type of ADCA. We also found two cases with 16q-linked ADCA among 43 sporadic patients with late-onset cortical cerebellar atrophy (LCCA). The mean age at onset in the 22 patients was 61.8 years, and that of homozygous patients was lower than that of heterozygous ones in one family. Neurological examination revealed that the majority of our patients showed exaggerated deep tendon reflexes in addition to the cardinal symptom of cerebellar ataxia (100%), and 37.5% of them had sensorineural hearing impairment, whereas sensory axonal neuropathy was absent. The frequency of 16q-linked ADCA was about 1/10 of our series of 110 ADCA families, making it the third most frequent ADCA in Japan.

Age of Onset↗

Acute ophthalmoplegia with pupillary areflexia associated with anti-GQ1b antibody.

Raised anti-GQ1b antibody is associated with Miller Fisher syndrome, Guillain-Barre syndrome (GBS) with ophthalmoplegia, Bickerstaff's brain stem encephalitis, acute ophthalmoparesis without ataxia and ataxic GBS without opthalmoplegia. We report a rare case of acute ophthalmoplegia associated with anti-GQ1b antibody that also had pupillary areflexia. A 35-year-old Chinese lady presented with external ophthalmoplegia, pupillary areflexia and no other abnormalities of cranial nerves, muscle tone, deep tendon reflexes, limb power or cerebellar dysfunction. Anti-GQ1b IgG antibody titre was significantly elevated, while neuroimaging of brain and orbital structures, nerve conduction study and cerebral spinal fluid examination were normal. Pupillary areflexia should be recognized as another feature that may be present in conditions associated with raised anti-GQ1b antibody.

Acute Disease↗

Prognostic predictors of thalamic hemorrhage.

BACKGROUND AND OBJECTIVE: There is no study evaluating the role of clinical, evoked potential and radiological parameters in the prognosis of thalamic hemorrhage employing multivariate logistic regression analysis, thus we aimed to evaluate the role of these parameters in predicting the 3 month outcome following thalamic hemorrhage. SETTING: Tertiary care referral teaching hospital. METHODS: Fifty-three patients with CT proven thalamic hematoma were evaluated. Conscious level was assessed using the Glasgow Coma Score (GCS), severity of stroke by the Canadian Neurological Scale (CNS), while muscle tone, tendon reflexes and power were also recorded. Hematoma size and type, and evidence of ventricular extension were obtained from the CT scan. Hematomas were classified as (A) thalamic with postero-lateral extension or (B) thalamic without postero-lateral extension. Central motor conduction to upper limb and median somatosensory evoked potentials (SEP) were recorded. Outcome was defined at 3 months on the basis of the Barthel Index (BI) with good being a BI of 12 or greater and poor a BI of less than 12. Best predictors of outcome were evaluated by single variable logistic regression analysis followed by multivariate logistic regression. RESULTS: Age ranged between 35 and 85 years; 18 were women. Mean GCS was 10.4 and CNS was 3.9. Thirty-one patients had type A hematomas and 22 type B. The hematoma was small in 5, medium in 35 and large in 13 patients. Ventricular extension was present in 34 patients. Motor evoked potentials were unrecordable in 36 and central motor conduction time was prolonged in 8 patients. Median SEP was unrecordable in 37 and N9-N20 conduction time was prolonged in 2 patients. At 3 months, 8 patients had died, 24 had good and 21 had poor outcome. On univariate logistic regression analysis diabetes mellitus, GCS, pupillary asymmetry, CNS score, type and size of hematoma and motor and somatosensory evoked potentials were significant in relation to outcome. On multivariate logistic regression analysis, the best predictors of outcome at 3 months were the type of hematoma and CNS score. CONCLUSION: CNS score and CT appearance of hematoma are the best predictors of 3 month outcome following thalamic hemorrhage. The proposed model for outcome assessment is simple and easy to apply and could have wide clinical application.

Adult↗

Clinical and electrophysiological characteristics of autosomal recessive axonal Charcot-Marie-Tooth disease (ARCMT2B) that maps to chromosome 19q13.3.

Charcot-Marie-Tooth disease (CMT) comprises a heterogeneous group of hereditary motor and sensory peripheral neuropathies. The autosomal recessive axonal form of CMT (ARCMT2) is rare. Eight patients of a large consanguineous family of Spanish ancestry in Costa Rica were diagnosed with ARCMT2B; previous genetic studies of this family revealed linkage to chromosome 19q13.3. The clinical and electrophysiological features of these patients are reported. All patients presented with a symmetric motor and sensory neuropathy, which was more pronounced in the lower limbs. Further, distal muscle wasting and impaired deep tendon reflexes were found. Age at onset was between 26 and 42 years, and the disease duration ranged from 2 to 19 years. Electrophysiological studies revealed a primary axonal degenerative process. The clinical characteristics of this family differed in several aspects from previously reported families with ARCMT2.

Action Potentials↗

A case of severe congenital chronic inflammatory demyelinating polyneuropathy with complete spontaneous remission.

Chronic inflammatory demyelinating polyneuropathy (CIPD) is characterised by progressive weakness, hyporeflexia and electrophysiological evidence of demyelination with maximal neurological deficit reached after at least 8 weeks progression. CIPD rarely affects children. We present a neonate with clinical features compatible with congenital CIPD. A term male infant of non-consanguineous parents was referred to us at birth with weakness and contractures affecting his legs, suggesting a prenatal onset of immobility. He also had evidence of bulbar dysfunction with poor suck, recurrent aspiration and requiring nasogastric feeding. He had no antigravity movements in the legs, bilateral wrist drop, distal joint contractures and absent deep tendon reflexes. Electromyography showed neurogenic changes, with nerve conduction velocities markedly reduced, increased distal motor latency and dispersed compound muscle action potentials. Cerebrospinal fluid protein was raised. Sural nerve biopsy demonstrated decreased numbers of myelinated fibres and inflammatory cell infiltrates. Muscle biopsy showed denervation. He only received supportive treatment and by 6 months he had fully recovered, and all electrophysiological parameters had normalised.

Disease Progression↗

Guillain-Barré syndrome.

Guillain-Barré syndrome (GBS) is an acute, demyelinating polyneuropathy involving the spinal roots, peripheral nerves, and often the cranial nerves. Although its exact mechanism remains unclear, an autoimmune etiopathology is theorized. It is characterized by rapidly progressing, symmetrical muscular weakness starting in the legs and ascending to the trunk and arms. Additionally, deep tendon reflexes are lost. Approximately half of the patients with GBS have cranial nerve palsies, with unilateral or bilateral facial nerve (CN VII) palsy being the most common. Paralysis of the muscles of the tongue, lips, palate, larynx, and pharynx from lesions involving cranial nerves IX, X, and XI and weakness of the muscles of mastication (CN V) are the next most common cranial nerve abnormalities. Ocular muscle palsy is not common, only occurring in approximately 10% of patients. The Miller Fisher variant of GBS is a distinct syndrome in which the only neurologic deficits are oculomotor palsies, areflexia, and ataxia. We present the rare case of a 45-year-old woman with GBS whose ocular muscles were affected. The oculomotor disturbance and limb weakness occurred within a few days of one another. Basic anatomic considerations are reviewed along with the pathophysiology and clinical features of GBS.

Diagnosis, Differential↗

Rapidly progressive spinal muscular atrophy in an ambulatory 2-year-old male.

A 2-year 9-month-old male was referred for gait disturbances. Main complaints were abnormal gait with frequent falls observed as soon as he began to walk unaided, at 18 months of age. The first neurologic examination revealed symmetric and proximal weakness in the lower limbs with difficulty running and walking upstairs. Deep tendon reflexes were decreased, and generalized hypotonia was observed. Three months later, at 3 years of age, he had lost independent gait, and 1 month later he could not stand unaided. DNA analysis revealed homozygous deletion in exons 7 and 8 of SMN1 gene, confirming the diagnosis of spinal muscular atrophy. According to the current classification, this patient would be classified as spinal muscular atrophy type III. The distinctive feature of this case was the short time elapsed (18 months) between onset of spinal muscular atrophy and the age at which he lost ambulation. This patient reinforces the notion that late onset of symptoms in spinal muscular atrophy and acquisition of independent gait do not exclude a rapidly progressive motor deterioration, which is important when talking with families about outcome. In those rapidly progressive cases, when promptly available, testing for SMN1 gene will prevent unnecessary, invasive, or uncomfortable procedures such as lumbar puncture, electromyography, or spinal cord magnetic resonance imaging.

Child, Preschool↗

Hereditary hypotonia, muscle weakness, failure to thrive, and cognitive delay in a large moslem kindred.

Five infants of a Moslem-Arab extended family were evaluated for common and characteristic clinical findings of failure to thrive, extreme muscle weakness, severe motor delay, and moderate to severe cognitive and verbal delay. All children were below the third percentile in weight and height, and three of them had head circumference below the third percentile. Neurologic examination revealed severe hypotonia, muscle weakness, and absent deep tendon reflexes. Two children died at 2 years of age, and none of the children acquired full head control and the motor milestones of rolling and sitting. Laboratory evaluation including muscle biopsies, genetic studies, and metabolic evaluation was nondiagnostic.

Biopsy↗

Acute arm paresis with cervical spondylosis: three case reports.

BACKGROUND: The mild type of anterior spinal artery syndrome (ASAS) is characterized by motor loss with an absent or insignificant sensory deficit due to a disturbance in the blood supply to the anterior horn of the spinal cord. The clinical symptoms of cervical spondylotic amyotrophy (CSA) are motor loss or atrophy with an absent or insignificant sensory deficit or a long tract sign; however, the pathophysiology has not been clarified. METHODS: Three patients who suffered from palsy of the deltoid and biceps brachii are presented. Magnetic resonance imaging confirmed the intrinsic cord disease as the cause of the paresis. We measured the central motor conduction time (CMCT) and the latencies of the tendon reflex (T waves) of the biceps and triceps and those of the F waves of the abductor pollicis brevis and abductor digiti minimi before, 2 weeks after, and 3 months after starting intravenous injections of prostaglandin E(1) (PGE(1)). RESULTS: In these 3 cases, restoration of muscle strength began after starting injection of PGE(1). The electrophysiologic diagnosis revealed a disturbance of the motor conduction, in the CMCT and the latencies of the T waves, in the paretic muscle, which is more severe than that in other muscles. The radiological diagnosis suggested damage in the spinal cord. Improvements in the disturbance of the motor conduction and those of symptoms were parallel. CONCLUSION: From symptomatologic or radiological viewpoints, it is difficult to differentiate CSA from ASAS with cervical spondylosis. This suggests that there have been patients with ASAS whom we have diagnosed as CSA, and we may add administration of PGE(1) to the treatment for the patients with CSA. The present 3 patients showed improvement of muscle strength after starting injections of PGE(1). Although this improvement was measured by an electrophysiologic method, the mechanisms of PGE(1) require further study.

Acute Disease↗

Extranodal Rosai-Dorfman disease with multiple spinal lesions: a rare presentation.

BACKGROUND: Rosai-Dorfman disease is an idiopathic histiocytic proliferative disorder characterized by sinus histiocytosis and massive lymphadenopathy. Extranodal involvement is rare and central nervous system (CNS) involvement is unusual. We present a case of a multiple-level spinal involvement by Rosai-Dorfman disease without cervical lymphadenopathy, which is distinctly rare. Very few cases have been reported in literature. Its resemblance with meningiomas both clinically and radiologically makes its diagnosis all the more difficult. CASE DESCRIPTION: A 23-year-old woman presented with quadriparesis and incontinence of urine and stool with a history of being operated on twice for dorsal spine tumor. On examination she had hypoesthesia below the C6 vertebra with anesthesia below D3, and absent joint position and vibration sensations. The patient had spasticity in lower limbs with normal tone in upper limbs. Deep tendon reflexes were hyporeflexic in upper limbs and hyperreflexic in lower limbs with bilateral ankle clonus with bilateral Babinski sign. Magnetic resonance imaging of the cervicodorsal spine was suggestive of multiple intradural extramedullary space-occupying lesion with probability of meningiomas. Surgical resection of 3 tumors was done and sent for histopathology, which was suggestive of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). The patient had good postoperative recovery and was followed up for 5 months with no new complaints. CONCLUSION: Rosai-Dorfman disease is a rare entity having clinical and radiological similarity to meningioma. It is similar to lymph node histiocytosis and rarely involves the CNS. It is a benign lymphohistiocytic proliferative condition of unknown etiology. Histopathology showing emperipolesis (lymphophagocytosis) and immunohistochemistry showing S-100 positivity is diagnostic. Surgical resection is the treatment of choice.

Adult↗

Congenital insensitivity-to-pain with anhidrosis (CIPA): a case report with 4-year follow-up.

Congenital Insensitivity-to-pain with anhidrosis (CIPA) is a rare disorder in which pain perception is absent from birth, despite the fact that all other sensory modalities remain intact or minimally impaired and tendon reflexes are present. The challenge in dentistry is to manage the self-mutilation behavior avoiding serious damages especially to oral structures, hands and fingers. A Brazilian case of CIPA is presented and discussed with clinical documentation of the oral-related problems over a 4-year follow-up. A conservative treatment (mouthguard-like appliance) was proposed with the objective to avoid full mouth extraction.

Consanguinity↗

Behr's syndrome and 3-methylglutaconic aciduria.

We examined three patients from two families of Jewish-Iraqi origin who had progressive reduction of visual acuity and childhood onset of bilateral optic nerve atrophy without additional retinal abnormalities. They had neurologic symptoms compatible with Behr's syndrome. Neurologic signs included increased tendon reflexes, a positive Babinski sign, progressive spastic paraplegia, dysarthria, head nodding, and horizontal nystagmus. Neurologic involvement varied between affected siblings. The patients excreted excessive amounts of 3-methylglutaconic acid and 3-methylglutaric acid in their urine. We compared the characteristic ophthalmic features and the spectrum of neurologic signs encountered in this recently delineated autosomal recessive clinical entity with those of previously described entities associated with 3-methylglutaconic aciduria. Patients with early-onset optic atrophy should be examined for neurologic signs and screened for organic aciduria. A detailed ophthalmic examination is important in patients with neurologic abnormalities compatible with Behr's syndrome.

Child↗

Gabapentin effect on spasticity in multiple sclerosis: a placebo-controlled, randomized trial.

OBJECTIVE: To investigate the effect of gabapentin on subject self-report and physician-administered spasticity scales in individuals with multiple sclerosis. DESIGN: Prospective, double-masked, placebo-controlled, crossover design. SETTING: The Multiple Sclerosis Center at the Denver Veterans Affairs Medical Center. INTERVENTION: Subjects were titrated to either 900 mg gabapentin orally three times a day or placebo over a 6-day period. Subjects underwent a 14-day washout and then were crossed over. No other changes were made to their medication regimen. MAIN OUTCOME MEASURES: The outcome measures were divided into two categories: subject self-report scales physician-administered scales. Subject self-report scales included the spasm frequency scale, spasm severity scale, interference with function scale, painful spasm scale, and global assessment scale. Physician-administered scales included the Modified Ashworth Scale, clonus scale, deep tendon reflexes, plantar stimulation response, and the Kurtzke Expanded Disability Status (EDSS) Scale. Digit Span and Digit Symbol subtests of the WAIS-R Intelligence Scale were administered to assess for possible impaired concentration. The Fatigue Impact Scale was administered to assess for changes in fatigue. The adjective generation technique was administered to assess for alterations in mood. RESULTS: A statistically significant reduction in the impairment of spasticity was found in the gabapentin-treated subjects compared with placebo as measured by the self-report scales of the spasm severity scale, interference with function scale, painful spasm scale, and global assessment scale and by the physician-administered scales of the Modified Ashworth and plantar stimulation response. No significant difference was noted in the Digit Span, Digit Symbol, adjective generation technique, and EDSS. CONCLUSION: Gabapentin reduces the impairment of spasticity, compared with placebo, without the side effects of worsening concentration and fatigue.

Acetates↗

Prospective study on the use of bolus intrathecal baclofen for spastic hypertonia due to acquired brain injury.

OBJECTIVE: To determine if the intrathecal delivery of baclofen will decrease spastic hypertonia caused by brain injury. PATIENTS: Eleven patients more than 1 year after their brain injury with disabling lower extremity spastic hypertonia. SETTING: University tertiary care outpatient rehabilitation clinic. DESIGN: Patients were a consecutive sample randomized in a double blind, placebo-controlled crossover study. INTERVENTIONS: Bolus intrathecal injection of either normal saline or 50 micrograms baclofen. MAIN OUTCOME MEASURES: Data for Ashworth rigidity scores, spasm scores, and deep tendon reflex scores were collected for both the upper extremities (UE) and lower extremities (LE). Changes over time were assessed via Friedman's test. Differences between the placebo and active drug at any given time were assessed via Wilcoxon signed-rank. RESULTS: Four hours after injection with the active drug (maximum effect) the average LE Ashworth score decreased from 4.2 +/- 0.8 (SD) to 2.2 +/- 0.6 (p = .0033), spasm score from 3.1 +/- 1.0 to 1.0 +/- 0.7 (p = .0032), and reflex score from 3.3 +/- 0.5 to 1.0 +/- 1.3 (p = .0033). The average UE Ashworth score decreased from 3.3 +/- 1.3 to 1.9 +/- 0.8 (p = .0033), spasm score from 1.8 +/- 1.3 to 0.6 +/- 1.0 (p = .007), and reflex score from 2.7 +/- 0.5 to 1.7 +/- 0.6 (p = .0111). No trend was observed over time with placebo administration. There were significant reductions in the average for LE Ashworth (p < .0001), spasm (p < .0001), and reflex (p < .0001) scores and for UE Ashworth (p < .0001) and spasm (p < .0004) scores observed over 4 hours (maximum effect) with active drug administration. No significant differences were noted between the active drug and placebo groups before administration in LE and UE Ashworth, spasm, or reflex scores. There were significant differences between the active drug and placebo groups at 4 hours after administration for LE and UE Ashworth, spasm, and reflex scores (p < or = .0272). CONCLUSION: Intrathecal injection of baclofen is capable of reducing the spastic hypertonia associated with brain injury.

Adult↗

Long-term continuously infused intrathecal baclofen for spastic-dystonic hypertonia in traumatic brain injury: 1-year experience.

OBJECTIVE: To determine if the long-term use of continuously infused intrathecal baclofen (ITB) over a 1-year period will control spastic-dystonic hypertonia in patients with traumatic brain injury (TBI). SETTING: Tertiary care outpatient and inpatient rehabilitation center directly attached to a university hospital. SUBJECTS: Persons with TBI and intractable spasticity and dystonia for more than 6 months' duration recruited in a consecutive manner. DESIGN: TBI patients were admitted to the study after screening via a bolus injection of either intrathecal normal saline or 50 microg of baclofen. Data for Ashworth rigidity scores, spasm scores, and deep tendon reflex scores were collected for both the upper extremities (UE) and lower extremities (LE). Patients whose LE Ashworth scores decreased an average of 2 points were then offered implantation of a computer-controlled pump for continuous ITB. Changes over time were assessed statistically via Friedman's analysis for ordinal data and ANOVA for linear data. Differences between set points in time were also assessed via Wilcoxon signed rank. DATA SET: Seventeen patients (average age 29+/-11 yrs) with spasticity and/or dystonia treated over 1 year via a computer-controllable intrathecal delivery system for the delivery of ITB. RESULTS: After 1 year of continuous ITB treatment the average LE Ashworth score decreased from 3.5+/-1.3 (SD) to 1.7+/-0.9 (p < .0001), spasm score from 1.8+/-1.3 to 0.2+/-0.5 (p< .0001), and reflex score from 2.5+/-1.1 to 0.1+/-0.3 (p < .0001). The average UE Ashworth score decreased from 2.9+/-1.5 to 1.6+/-1.0 (p < .0001), spasm score from 1.2+/-1.5 to 0.2+/-0.6 (p < 0.0001), and reflex score from 2.2+/-0.5 to 1.0+/-0.8 (p < .0001). The average ITB dose required to attain these effects at 1 year was 302 microg continuously infused per day. CONCLUSION: Continuous intrathecal infusion of baclofen is capable of maintaining a reduction in spasticity and dystonia in both the upper and lower extremities of TBI patients.

Adolescent↗