Spastic Quadriparesis Research Articles

Cerebral palsy lifetime care – four musculoskeletal conditions

Cerebral palsy (CP) has always been considered a static condition in the neurological sense. Secondary and associated conditions that occur in the patient with CPcan progress over time and cause unwanted sequelae. This paper discusses four musculoskeletal conditions that present across the lifetime and can lead to progressive loss of function in the patient with CP. Patella alta can be particularly painful in the early adult years, limiting mobility particularly when associated with crouch gait. Adults with lower-extremity weight-bearing status having hip dysplasia, progressive over time, often develop pain and severe degenerative arthritis, with or without arthrodesis. Spondylolysis, particularly at the L5 S1 level, is fairly common in the ambulatory adult with diplegia and may, if not diagnosed early, progress to spondylolisthesis. Cervical stenosis appears to be more prevalent in adults with spastic quadriparesis and dystonia and is often associated with myelomalacia and/or radiculopathy. All four of these conditions may be lessened, or even prevented, with intervention and diagnosis in the younger years. Possible interventions and outcomes over time are discussed in the context of multidisciplinary team management of the individual with CP.

Cervical intradural extramedullary ependymal cyst associated with congenital dermal sinus: a case report

Spinal intradural extramedullary ependymal cyst is a very rare entity with only few cases reported in the literature. Its association with congenital dermal sinus has not been described so far. We present a unique report of a 3-year-old male child who presented with spastic quadriparesis with a trophic ulcer in the right great toe of 1-year duration. He harbored a congenital dermal sinus in the cervical spine since birth. Intraoperatively, the sinus was associated with an intradural cyst which proved to be an ependymal cyst on histopathological examination. The clinical profile along with review of literature of this rare entity is presented.

“Anchor”-shaped bright posterior column in a patient with vitamin B12 deficiency myelopathy

A 50-year-old strict vegetarian lady presented with progressive gait instability and paraesthesia of all four limbs lasting 8 months. She had spastic ataxic quadriparesis with pan sensory loss below the...

Cervical Stenosis and Spastic Quadriparesis in Morquio Disease (MPS IV)

White, Klane K. MD, MSc1; Steinman, Suzanne MD2; Mubarak, Scott J. MD2 Author Information

Central Pontine Myelinolysis

A 26-year-old man with a history of chronic alcohol abuse presented with dysarthria, lethargy, and horizontal nystagmus. Results of a clinical examination and blood tests were otherwise normal. Spastic quadriparesis developed.

Progressive multifocal leukoencephalopathy in a patient with B-cell lymphoma during rituximab-containing chemotherapy: case report and review of the literature

Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease of the central nervous system caused by the JC polyomavirus. We describe a rare case of PML in a 48-year-old female patient with diffuse large B-cell lymphoma who received rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisolone (R-CHOP) therapy. While she was undergoing five cycles of R-CHOP, she noticed gradually progressive neurological symptoms, such as slurred speech and gait disturbance, and she eventually developed high-grade fever. She also developed Pneumocystis jiroveci pneumonia. The neurological symptoms deteriorated thereafter, and she developed spastic quadriparesis and bulbar palsy. Magnetic resonance imaging showed hyperintensity within the right cerebellar hemisphere on T2-weighted images. Polymerase chain reaction-based tests of the cerebrospinal fluid revealed the presence of the JC virus. Despite intravenous and intrathecal cytarabine treatment, the patient died of PML 5 months after it was diagnosed. Retrospective analysis of her laboratory data showed that her CD4(+) T-cell count before R-CHOP therapy had decreased to 68 microL(-1). Thus, when administering rituximab-containing chemotherapy, even to patients with no prior history of opportunistic infections, attention should be paid to the potential occurrence of PML, particularly in patients with low CD4(+) T-cell counts.

Treatment-Induced Plasticity in Cerebral Palsy: A Diffusion Tensor Imaging Study

Diffusion tensor imaging is used as a measure of white-matter organization to probe mechanisms underlying clinical responses. Diffusion tensor imaging and clinical assessment in 8 patients with spastic quadriparesis (mean age, 6.13 years) was performed before and 6 months after therapy (botulinum injection, followed by physiotherapy). All patients were graded on the basis of gross motor function. Serial diffusion tensor imaging was also performed on 10 age/sex-matched controls at baseline and after 6 months. Regions of interests were placed on corticospinal tracts at different levels (i.e., corona radiata, posterior limb of internal capsule, midbrain, pons, and upper medulla) and on other major white-matter tracts, in both patients and controls. A significant increase in fractional anisotropy was evident in corticospinal tracts at the level of the posterior limb of the internal capsule and periventricular white matter of the temporal lobe, relative to baseline values in the patient group. Gross motor function classification system grades improved in all patients during follow-up relative to baseline values. The increase in fractional anisotropy in corticospinal tracts, along with improved clinical motor scores, suggests plasticity of the central motor pathway after combined therapy.

Giant Congenital Melanocytic Nevus Coexistent with Chiari II Malformation

Giant congenital melanocytic nevus (GCMN) and Chiari II malformation (CM II) are distinct rare clinical entities. No previous report of this condition is to be found in the medical literature. We report the case of a male infant seen initially for a GCMN and an upper thoracic myelomeningocele. MRI incidentally revealed cerebellum and brain stem elongate via the foramen magnum. The infant's physical examination included spastic quadriparesis and hypotonia. An operation for myelomeningocele repair and suboccipital craniectomy with resection of C (1) and cerebellar tonsils was done. The signs and symptoms of the patient were improved after surgery. The GCMN was not treated according to the parents' wished, but a schedule of long-term monitoring was recommended. This case report illustrates that CMNs can coexist with CM II. The clinician who diagnoses a patient with CMNs should remember that this entity can be associated with other pathologies of the central nervous system. Earlier diagnosis and surgical intervention improves the prognosis. Long-term monitoring and follow-up should be recommended for patients with CMNs.

Tuberculous meningitis presenting with unusual clinical features in Nigerians: Two case reports

BackgroundTuberculous meningitis is common in developing countries and accounts for about 7.8% to 14% of all cases of tuberculosis in Nigeria.Case presentationCase 1 was a 17-year-old woman who presented with a 3-week history of weakness of the right upper and lower limbs, a 6-hour history of inability to speak and irrational behaviour. She had no remarkable past medical history. Physical examination revealed pyrexia (temperature of 38.2°C) and altered level of consciousness (Glasgow coma score = 7/15). The signs of meningeal irritation were present and she had anisocoria and right spastic hemiparesis. Other aspects of physical examination were normal. Laboratory investigations showed an elevated erythrocyte sedimentation rate, normal cerebrospinal fluid protein and reduced glucose. The brain computed tomography scan showed features in keeping with obstructive hydrocephalus and she was immediately commenced on antituberculous drugs, intravenous steroids and mannitol. She made a remarkable clinical recovery and was discharged home 6 weeks after admission. Case 2 was a 40-year-old man who presented with a 6-week history of headache and fever and a 2-week history of alteration in level of consciousness. There was no history of neck pain and/or stiffness, nausea or vomiting. He had no other remarkable past medical history. He had been placed on various intravenous antibiotics in private hospitals before presentation, with no clinical improvement. Physical examination showed a young man in a coma (Glasgow coma score = 4/15) and febrile (temperature of 38.5°C) with signs of meningeal irritation. The brain stem reflexes were impaired and he had spastic quadriparesis. Further physical examination was essentially normal. The cerebrospinal fluid analysis showed features in keeping with meningeal inflammation and he had a raised erythrocyte sedimentation rate. The brain computed tomography scan showed features in keeping with obstructive hydrocephalus. He was placed on antituberculous drugs and intravenous steroids but despite this his clinical condition deteriorated and he died on the sixth day after admission.ConclusionLate presentation of tuberculous meningitis is not rare in Nigerians and we report two cases of tuberculous meningitis that presented late to our health care facility. This report is intended to make clinicians aware of the unusual clinical presentations of tuberculous meningitis.

Fluorotic cervical myelopathy

A 65-year-old male presented with severe neck and low back pain for the past 6 months and progressive spastic quadriparesis for the past 15 days. All deep tendon reflexes were...

Cervicobulbar intramedullary lipoma

Background context Spinal lipomas not associated with spinal dysraphism are rare entities. Further, large medullary lipomas with intracranial extension are occasionally described. Most of the intradural lipomas are subpial and not really intramedullary. Purpose Clinicians may be made aware of such a rare entity and its presentation. Study design/setting An extremely rare case of medullary lipoma extending into posterior fossa is described in an adult, not associated with spinal dysraphism. Methods A young male presented with high cord myelopathy in the form of spastic quadriparesis. Radiological investigations revealed dorsally placed intramedullary lipoma extending into posterior fossa. Results Surgical excision of tumor provided satisfactory resolution of symptoms. Conclusions Medullary lipomas may present with features of high cord compression and myelopathy. Magnetic resonance imaging remains the investigative and follow-up modality of choice. Even when total tumor excision is not feasible, subtotal removal and decompression provides long lasting symptom-free survival.

A massive cervicomedullary intramedullary spinal cord lipoma

We present a large cervicomedullary intramedullary lipoma in a 17-year-old boy, who presented with spastic quadriparesis and exaggerated reflexes. MRI revealed an intramedullary lipoma extending from the craniovertebral junction to the sixth cervical vertebra. There was no spinal dysraphism. Subtotal excision with primary dural closure was performed, which produced an improvement in the patient’s condition. The technique we used is discussed.

Reversible posterior leukoencephalopathy in a patient with autonomic dysreflexia: a case report

Case report. To report a case of reversible posterior leukoencephalopathy (RPL) in a patient with traumatic cervical spinal cord injury. Neurologic inpatient Unit, Lahey Clinic, Burlington, MA, USA. A 55-year-old woman with a residual spastic quadriparesis from a traumatic C5-C6 fracture developed, during an assisted cough maneuver, sudden severe headache followed by cortical blindness in the setting of high blood pressure. Magnetic resonance imaging (MRI) showed T2 hyperintensities in the subcortical white matter of both medial occipital lobes and left post-central gyrus. Elevation of the head of the bed during subsequent cough maneuvers with occasional use of sublingual nifedipine prevented further episodes of acute arterial hypertension and development of new symptoms. Repeat MRI of the head done one month later was normal. RPL can occur in the setting of autonomic dysreflexia in patients with traumatic cervical cord injury. The prompt recognition of this syndrome is of importance to prevent further morbidity and mortality in patients with spinal cord injury.

Craniocervical developmental anatomy and its implications

Congenital and developmental osseous abnormalities and anomalies that affect the craniocervical junction complex can result in neural compression and vascular compromise and can manifest itself with abnormal cerebrospinal fluid dynamics. An understanding of the development of the craniocervical junction is essential to recognize the pathological abnormalities. Atlas assimilation, segmentation failures, os odontoideum, basilar invagination, and the various syndromes that affect the craniocervical junction have been analyzed. The natural history provides an added insight into its treatment. Proatlas segmentation abnormalities surrounded the foramen magnum and the posterior arch of C1. Hindbrain herniation was associated in 33 of the 90 children involved. Spastic quadriparesis presented in 80% and lower cranial nerve abnormalities in 33%. Vertebrobasilar dysfunction was observed in 40% and trauma presentation seen in 60% of individuals. Atlas assimilation was present in 550 individuals who were evaluated for craniovertebral junction abnormalities. Hindbrain herniation occurred in 38%. Segmentation failure of C2 and C3 vertebrae compounded the abnormal dynamics resulting in atlantoaxial instability. This was a reducible instability with formation of pannus around the odontoid process until it became irreducible at approximately 14 years of age. Unilateral atlas assimilation caused torticollis in children. Os odontoideum was investigated regarding craniocervical trauma at a young age. The conclusion was that os odontoideum was associated with an unrecognized fracture in children below the age of 5 with a previously normal odontoid structure as observed in our series. Atlas and axis abnormalities were reviewed in this series. This large database has provided an understanding of the natural history of many entities and allowed treatment protocols to be established that have stood the test of time.

Shunt revision after 33 years in a patient with bilateral calcified chronic subdural hematomas

cation of shunting procedures for infantile hydrocephalus.1,2,4 Bilateral calcified chronic SDHs may be so extensive that they give the appearance of a so-called “armored brain.”3,5 This 33-year-old man was admitted to our department with symptoms of increased intracranial pressure. According to his medical history, the patient had undergone a ventriculoatrial shunt insertion for hydrocephalus 2 months after his birth at a children’s hospital. His mild spastic quadriparesis and learning difficulties were obvious since childhood. One year before the present admission, a brain computed tomographic (CT) scan performed after a minor head injury had shown the presence of large, calcified, bilateral SDHs, together with small ventricles and a ventricular catheter in place (Fig. 1A). At admission, a new brain CT scan revealed ventricular dilation (Fig. 1B). A ventriculoperitoneal shunt was inserted in the patient. The new ventricular catheter was inserted via a right frontal bur hole near the midline, avoiding the calcified hematoma. The old shunt was left in place because the catheter was considered to be entrapped inside the calcification. The postoperative clinical course of the patient was uneventful. The patient improved and returned to his neurological status before the dysfunction of the shunt, and a postoperative CT scan confirmed a reduction of ventricular size (Fig. 1C). Chronic SDHs that remain undiagnosed during childhood may contribute to neurological impairments, therefore intensive followup is necessary after shunting procedures in infants and children. Cases of bilateral extensively calcified SDHs have been rarely reported.5 Revision of the malfunctioning shunt in cases like these may be troublesome. (DOI: 10.3171/JNS/2008/108/2/0401)

Congenital irreducible atlantoaxial dislocation associated with cervical intramedullary astrocytoma causing progressive spastic quadriparesis

Simultaneous presence of congenital irreducible atlantoaxial dislocation (AAD) and cervical intramedullary astrocytoma has not been previously described and may cause disabling myelopathy. This 55-year-old lady presented with suboccipital pain, spastic quadriparesis, Lhermitte's phenomenon and sphincteric disturbances. Lateral radiographs and magnetic resonance imaging showed irreducible AAD, occipitalized atlas, C2-3 fusion, and,an intramedullary tumor from C2-5 level iso-to-hypointense, non-enhancing, except in a small segment in the dorsal C2 level. A suboccipital craniectomy with C2-5 laminectomy revealed a greyish-white tenacious tumor. The tumor was decompressed using a C2-5 midline myelotomy and duroplasty. An occipitocervical lateral mass fixation was performed. Histopathology revealed a low-grade astrocytoma. At three-month follow-up, her spasticity had decreased and quadriparesis and sphincteric disturbances were persisting. Postoperative lateral radiographs and intrathecal contrast CT scan showed a stable occipitocervical construct. Thus, the suboccipital craniectomy and laminectomy with midline myelotomy and duroplasty facilitated space for progressively expanding intramedullary astrocytoma with irreducible AAD; the lateral mass fixation provided stability at the craniovertebral junction.

Upper Motor Neuron Syndrome Associated with Subclinical Sjoegren's Syndrome

We present two patients with primary lateral sclerosis-like upper motor neuron disease accompanying subclinical Sjögren's syndrome. Both patients showed progressive spastic quadriparesis, but neither sensory involvement nor detrusor dysfunction was noted. Lower motor neuron signs were detected only in their late follow-up period. Although sicca symptom was nearly absent, salivary labial gland biopsy revealed marked sialoadenitis in both patients. They also displayed a constellation of findings that suggested an autoimmune etiology closely related to Sjögren's syndrome, including germinal center formation in one patient, and markedly elevated levels of anti-nuclear antibody with abnormal sialography in the other. Both patients showed significant neurological improvement after the initial course of intravenous immunoglobulin therapy. We suggest that the evidence for subclinical Sjögren's syndrome should be sought in patients presenting with selective upper motor neuron involvement.

Iatrogenic Botulism in a Child With Spastic Quadriparesis

Botulinum toxins are potent neurotoxins used in a variety of neurological disorders. Few pediatric reports have been published to date regarding the potential hazards of therapeutic use of botulinum toxins. We describe the case of a 10-year-old boy who developed systemic weakness following treatment of spasticity with botulinum toxin type B. The patient developed iatrogenic botulism with ptosis, facial diplegia, neck flexor and extensor weakness, and profound hypopharyngeal laxity with respiratory compromise from which he eventually recovered. Clinicians should be mindful of the risk for systemic botulism when using local injections of the neurotoxin.

Poster 162: Changes in Bladder Function After Initiation of Intrathecal Baclofen in Patients With Supraspinal Spasticity: A Report of 2 Cases

Setting: Tertiary care hospital. Patients: 2 female patients, ages 30 and 15 years, with spastic quadriparesis, one due to cerebral palsy and one due to traumatic brain injury. Case Descriptions: Both patients have had pumps placed for intrathecal baclofen (ITB) administration to manage spasticity, had not had urinary problems associated with oral baclofen, and had a change in urinary status following ITB administration. Urodynamic studies were performed to assist with urinary management after pump implantation due to new onset urinary retention. Both had stable urinary management with infrequent urinary tract infections (UTIs). Assessment/Results: Urinary management prior to pump implantation was timed voiding with episodes of urinary incontinence, with low post void residuals. Both patients had frequent UTIs and urinary retention after pump implantation. Urodynamic studies showed detrusor sphincter dyssynergia with normal bladder compliance and poorly contracting detrusor. Intermittent catheterization was initiated, although it was not continued long term. Discussion: There are reports of the positive and negative effects of ITB on spinal-mediated neurogenic bladders. However, there are fewer reports about patients with supraspinal spasticity receiving ITB, and none reporting urodynamics. There are several mechanisms for urinary dysfunction, and ITB may have variable effects on some of them. In the patients reported here, new onset urinary retention may be a factor in a decreased micturition reflex, resulting in normal detrusor compliance but ineffective detrusor contraction. There appeared to be little ITB effect on pelvic floor muscle activity. Conclusions: Urinary function can change following initiation of ITB in patients with supraspinal spasticity. Documentation of urinary function preimplant is important for long-term management, and urinary function should be monitored after ITB initiation. Urodynamics are useful for diagnosis and treatment if bladder function changes occur.

The long-term outcome in surviving infants with Apgar zero at 10 minutes: a systematic review of the literature and hospital-based cohort

The purpose of this study was to investigate the outcomes of infants who had Apgar scores of zero at 10 minutes and were resuscitated successfully. The literature was reviewed systematically to identify the outcomes of cases; in addition, the perinatal database at the John Radcliffe Hospital, Oxford, was used to identify similar cases. Eligible infants were identified through hospital records, and outcomes of all infants with an Apgar score of zero at 10 minutes, who were born between January 1991 and December 2004, were reviewed. Eighty-five cases were identified from the literature. With the Oxford database, 9 of 83,065 infants (0.12/1000 births) met our study criteria. Six of the 9 infants died before leaving hospital. One infant with severe quadriparesis and microcephaly died at 11 months of age. One infant at follow-up examination at 5 years had severe spastic quadriparesis with severe global delay. One infant with grade 2 hypoxic-ischemic encephalopathy, who was born with severe anemia that was corrected promptly at birth, had mild disability at follow-up examination at 2 years. Thus, death or severe disability occurred in 8 of 9 infants. Combining the results of metaanalysis of published data with our results of 94 infants, 88 infants (94%) either died or were handicapped severely; 2 infants (2%) were handicapped moderately, and 1 infant (1%) was handicapped mildly. For 3 infants (3%), the long-term outcome could not be determined. The outcome of infants with an Apgar score of zero at 10 minutes is almost universally poor.