Paraneoplastic cerebellar degeneration and Horner syndrome: association of two uncommon findings in a child with Hodgkin disease

An 11-year-old boy admitted with a right cervical mass was found to have Hodgkin disease. On admission, he also had right Homer syndrome and severe cerebellar ataxia. Cranial MRI revealed marked cerebellar atrophy. He was treated with chemotherapy consisting of doxorubicin, bleomycin, vinblastine, and dacarbazine (ABVD), in addition to radiotherapy. Three months after initiation of therapy, he had a partial remission of tumor. Neurologic symptoms improved dramatically after chemotherapy started. Hodgkin disease should be included in the differential diagnosis of children with cerebellar findings and Horner syndrome.     

Cerebellar vincristine toxicity (author's transl)]

The diagnosis of Wilms tumor stage IV was established in a girl of 5 1/2 years of age in July 1972. Nephrektomy was followed by radiotherapy of the tumorbed and the lung metastasis. Thereafter the child was treated with Vincristine (VCR) and Actinomycine D (AMD) for two years. The child has been off treatment for the last 14 months and is free of disease. An intention tremor of the right hand was noted 4 weeks after the 32nd application of VCR and 2 weeks after a viral infection of unknown etiology. The writing proved to be hypermetric. There was no loss of proprioception and thus the symptoms were localized into the right cerebellum. Having excluded other causes (hemorrhage, metastasis) as far as possible and considering unilateral cerebellar encephalitis as very unlikely the cerebellar symptoms were thought to be VCR toxicity.     

Effectiveness of a Co-Taught Handwriting Program For First Grade Students

ABSTRACT

Our study examined the effects of Write Start, a classroom-embedded handwriting/writing program on handwriting and writing fluency for first grade students, co-taught by occupational therapists and teachers. Two first grade classrooms received the Write Start and two received standard handwriting instruction. This co-taught program included specific feedback during handwriting practice, small group activities, student self-evaluation, and peer supports. The students were evaluated on handwriting legibility, fluency, and written expression at baseline, immediately after the program, and 6 months later. When performance was compared between the two groups, the students in the Write Start program improved significantly more in legibility (d = .57) and fluency (d = .75) than students who received standard instruction. Gains in handwriting speed (d = .18), average legibility (d = .26), and written expression (d = .25) did not differ significantly between the two groups. A co-taught, inclusive handwriting/writing program can promote first grade students’ achievement of lower case legibility and writing fluency.     

Comparison of Cursive Handwriting Instruction Programs Among Students Without Identified Problems

The purpose of this study was to compare the effects of cursive handwriting programs in improving letter legibility and form in third-grade students without identified handwriting problems. Four months into the school year, cursive handwriting was assessed for a sample of convenience of 50 third-grade students. Subsequently, students received instruction for 10–15 minutes daily for 6 weeks using either Handwriting without Tears, Loops and Other Groups programs, or, as a control condition, continued instruction in the Zaner-Bloser program. Student handwriting for all programs improved from pretest to posttest. Posttest comparisons indicated no significant differences between programs. The results suggest that the method of handwriting instruction has a limited short-term impact on cursive letter legibility and form for children without handwriting problems.     

Effectiveness of a computer skills program to improve written communication in children with Developmental Coordination Disorder

Word processing using a keyboard is an option for children with Developmental Coordination Disorder (DCD) who have printing/ handwriting difficulties. A five-replication single-subject ABA design was used to explore the ability of 6 children with DCD (entering Grades 3-5) experiencing problems with printing/handwriting to learn computer skills. Following a two-week intervention all children were familiar with basic computer functions and showed improved word processing skills. Touch-keyboarding speed increased for the five children entering Grades 4-5, but only one child acquired a speed (letters/minute) comparable to printing/ handwriting. Four children demonstrated increased text production when generating a story, but none achieved speeds comparable to printing/handwriting. Five-finger touch-keyboarding proficiency was better for children in Grades 4-5. Therapists observed improved legibility and increased motivation. Results suggest that the child's grade, program support, and program length should be considered when making decisions about keyboarding.     

Traumatic bilateral vertebral artery dissection at the dural entry point site in a 10-year-old boy

We present a 10-year-old boy who was admitted with headache and neurological symptoms after a trauma in the schoolyard. Cerebral MRI revealed an extensive ischaemia in the bilateral cerebellar hemispheres, left middle cerebellar peduncle, and right vermis. Digital substraction angiography demonstrated bilateral vertebral artery dissections at the dural entry point site. This case emphasises the management of patients with traumatic vertebral artery dissection.     

Family-Centred Care: A Critical Perspective

ABSTRACT

Purpose: Pain associated with hypermobility of wrist and hand joints can contribute to decreased handwriting output. This study examined the effectiveness of a neoprene wrist/hand splint in reducing pain and increasing handwriting speed and endurance for students with joint hypermobility syndrome. Methods: Multiple baseline, single system design (SSD) methodology was used. Four ninth grade students with handwriting difficulties because of joint hypermobility syndrome participated in this study. Results: Visual and statistical (two standard deviation band method) analyses indicated a significant decrease in handwriting speed when using the splint for three out of four participants. No significant change in pain or endurance was noted during intervention. There was a significant decrease in pain following withdrawal of the splint for three participants. Conclusion: Evidence from this study does not support use of this particular splint for decreasing pain and increasing handwriting speed and endurance for ninth grade students with joint hypermobility syndrome.     

Cerebellar vermis hypoplasia with extracerebral involvement (retina, kidney, liver): difficult to classify syndromes]

Congenital cerebellar vermis hypoplasias diversely associated with retinopathy, nephropathy and hepatopathy are rare syndromes of uncertain nosology. We report three new cases. CASE REPORTS: Case 1. A 3-month-old boy presented a brief nystagmus. At the age of 2 years, he had facial dysmorphia, hypotonia, ataxia, ocular motor apraxia and neurodevelopmental impairment with cerebellar vermis hypoplasia. The electroretinogram showed asymptomatic retinal involvement. At the age of 6 years, he developed chronic renal failure. The diagnosis of familial juvenile nephronophthisis was made by detection of a large homozygous deletion of the NPH1 region. Case 2. A term newborn boy presented apnea, tachypnea, hypotonia, nystagmus, ptosis, lack of visual contact and hepatomegaly. He had facial dysmorphia, bilateral optic coloboma with chorioretinal dysplasia and cerebellar vermis hypoplasia. There were cysts in the kidneys with increased echogenicity and lack of demarcation between the pyramids and the cortex. The liver was hyperechoic with fibrosis. At the age of 15 months, the child had severe developmental delay. He had bouts of fever. A search for a large homozygous deletion of the NPH1 region was negative. Case 3. A term newborn girl presented difficulty to suck, cyanosis, hypotonia and ptosis. Later, the child had a developmental delay. At the age of 6 years, she developed chronic renal failure (nephronophthisis). At the age of 23 years, she presented divergent strabismus, ataxia, mental retardation, slow ocular pursuit and facial dysmorphia. The neuroimaging showed a cerebellar vermis hypoplasia. A search for a large homozygous deletion of the NPH1 region was negative. CONCLUSION: The diagnosis of cerebellar vermis hypoplasia requires searching for retina, kidney and liver involvement. The large homozygous deletion of the NPH1 region has to be investigated if typical familial juvenile nephronophthisis is associated. Because cerebellar vermis hypoplasia with extracerebral involvements (retina, kidney, liver) is part of many different closely related syndromes, a clear molecular classification is necessary for accurate genetic counselling and an early prenatal diagnosis.     

Analysis of handwriting of children during treatment for acute lymphoblastic leukemia

BACKGROUND: Children treated for acute lymphoblastic leukemia (ALL) often complain about handwriting problems. PROCEDURE: Using a computerized writing task, we have prospectively studied the processes necessary for the production of handwriting movements in 11 children (5-12 years old) during treatment for ALL. Children were tested at time points closely related to the vincristine administration. RESULTS AND CONCLUSIONS: Children treated for ALL drew slower, with longer pause durations and increased drawing pressure. Children were able to overcome the problems, except for a consistently increased drawing pressure. This increased drawing pressure may be an attempt of the children to obtain sufficient kinesthetic information and thus can be seen as an adequate adaptation mechanism in case of peripheral neuropathy due to the neurotoxic effects of vincristine. However, neurotoxic effects of other cytostatic drugs cannot be excluded.     

Keyboarding for Students with Handwriting Problems

A literature review is presented regarding keyboarding for school students experiencing handwriting difficulties. Despite the overall dearth of research, some general conclusions appear warranted. Students need to be able to keyboard at least as fast as they can handwrite and should learn the touch-keyboarding method if possible. Appropriate instruction appears critical for the development of keyboarding competency. The upper elementary age is an appropriate time to start teaching keyboarding, with students possibly requiring 25-30 total hours of instruction. Students experiencing handwriting difficulties might need customized goals and strategies. Although the existing literature regarding the role of performance components in keyboarding provides some direction to clinicians, further investigation is required.     

Comparison of the Properties of the Handwriting Speed Test (HST) and Detailed Assessment of Speed of Handwriting (DASH): An Exploratory Study

Background: Handwriting speed is an important component of students’ ability to adequately express their ideas, knowledge and creativity in a timely and effective manner. Aims: Psychometric properties of the Handwriting Speed Test (HST) and Detailed Assessment of Speed of Handwriting (DASH) and accuracy of the norms for identifying current Australian students with handwriting speed difficulties were examined. Methods: An exploratory, cross-sectional study was conducted involving students, with and without handwriting difficulties, in Years 3–12 (mean age: 12.0 yrs, SD = 3.0 yrs; range = 7 to 18 yrs) in New South Wales (NSW; Australia). Participants were recruited through occupational therapists and schools. Students completed the HST and all DASH subtests. Results: Thirty-two students with, and 139 students without, handwriting difficulties participated. Intra-rater and inter-rater reliability were found to be excellent; sensitivity was low and specificity high for the HST and DASH. No significant differences were found between test scores and normative data for students without handwriting difficulties (year/age groups with n > 10). Conclusions: The HST and DASH are reliable assessments of handwriting speed. Further research is required into discriminant validity of the HST and DASH and need for updated norms.     

Keyboarding for students with handwriting problems: a literature review

A literature review is presented regarding keyboarding for school students experiencing handwriting difficulties. Despite the overall dearth of research, some general conclusions appear warranted. Students need to be able to keyboard at least as fast as they can handwrite and should learn the touch-keyboarding method if possible. Appropriate instruction appears critical for the development of keyboarding competency. The upper elementary age is an appropriate time to start teaching keyboarding, with students possibly requiring 25-30 total hours of instruction. Students experiencing handwriting difficulties might need customized goals and strategies. Although the existing literature regarding the role of performance components in keyboarding provides some direction to clinicians, further investigation is required.     

Relationships between fine-motor, visual-motor, and visual perception scores and handwriting legibility and speed

Occupational therapists assess fine motor, visual motor, visual perception, and visual skill development, but knowledge of the relationships between scores on sensorimotor performance measures and handwriting legibility and speed is limited. Ninety-nine students in grades three to six with learning and/or behavior problems completed the Upper-Limb Speed and Dexterity Subtest of the Bruininks-Oseretsky Test of Motor Proficiency, the Beery-Buktenica Developmental Test of Visual-Motor Integration-5th Edition, the Test of Visual Perceptual Skills-Revised, the Visual Skills Appraisal, and a handwriting copying task. Correlations between sensorimotor performance scores and handwriting legibility varied from .07 to .38. Correlations between sensorimotor performance scores and handwriting speed varied from .04 to .42. Stepwise multiple regression analysis indicated that the variance in handwriting explained by these measures was ≤ 20% for legibility and ≤ 26% for speed. On the basis of multivariate analysis of variance only scores for the Developmental Test of Visual-Motor Integration differed between students classified as "skilled" and "unskilled" handwriters. The low magnitude of the correlations and variance explained by the sensorimotor performance measures supports the need for occupational therapists to consider additional factors that may impact handwriting of students with learning and/or behavior problems.     

Handwriting Performance of Children with Attention Deficit Hyperactive Disorders: A Pilot Study

Objective. The aim of this study was to compare the handwriting characteristics of children diagnosed with attention deficit hyperactive disorder (ADHD), while on and off medication, as well as with a control group. Methods. Twelve children with ADHD and 12 control children, aged 8–10, performed a paragraph-copying task using a computerized system. The children with ADHD performed the task both while on and off medication. The handwriting product of both groups was then evaluated with the Hebrew Handwriting Evaluation tool. Conclusion. Results provide evidence for poorer performance of children with ADHD in comparison to children without ADHD on most handwriting process and product measures. Children with ADHD demonstrated significantly more total time including in-air time spent in handwriting performance when off medication. The possible implications of these results regarding the future use of handwriting process and product evaluations for children with ADHD in school settings are discussed.     

Relationships Between Fine-Motor,Visual-Motor,and Visual Perception Scores and Handwriting Legibility and Speed

ABSTRACT

Occupational therapists assess fine motor, visual motor, visual perception, and visual skill development, but knowledge of the relationships between scores on sensorimotor performance measures and handwriting legibility and speed is limited. Ninety-nine students in grades three to six with learning and/or behavior problems completed the Upper-Limb Speed and Dexterity Subtest of the Bruininks–Oseretsky Test of Motor Proficiency, the Beery–Buktenica Developmental Test of Visual-Motor Integration—5th Edition, the Test of Visual Perceptual Skills—Revised, the Visual Skills Appraisal, and a handwriting copying task. Correlations between sensorimotor performance scores and handwriting legibility varied from .07 to .38. Correlations between sensorimotor performance scores and handwriting speed varied from .04 to .42. Stepwise multiple regression analysis indicated that the variance in handwriting explained by these measures was ≤20% for legibility and ≤26% for speed. On the basis of multivariate analysis of variance only scores for the Developmental Test of Visual-Motor Integration differed between students classified as “skilled” and “unskilled” handwriters. The low magnitude of the correlations and variance explained by the sensorimotor performance measures supports the need for occupational therapists to consider additional factors that may impact handwriting of students with learning and/or behavior problems.     

Acute hydrocephalus drained in emergency. Consequence of cerebellar infarction in Haemophilus meningitis

A 2 year-old child admitted for Haemophilus meningitis was immediately treated by adequate antibiotic treatment. Three days later multiple hypertonic strokes and periodic respiration occurred; a resuscitation was necessary. CAT scan showed an acute hydrocephalus with non visible 4th ventricle and low-density areas in both cerebellar hemispheres allowing the diagnosis of cerebellar infarction. External drainage of CSF was rapidly performed and maintained for 11 days with success. The child was secondarily discharged with temporary cortical blindness and persistent moderate static cerebellar signs. The etiology of the cerebellar infarction was likely to be an arterial thrombosis in the vertebro-basilar area, probably secondary to cerebral arteritis related to Haemophilus.     

Primary tuberculous cerebellar abscess: case report

Tuberculous cerebellar abscess is a rare manifestation of central nervous system tuberculosis. An 8-year-old boy is described who presented with acute hydrocephalus and right hemiparesis owing to a cerebellar abscess.     

The neurobiology of attention deficit/hyperactivity disorder

ADHD is a brain based disorder with structural and functional abnormalities in widespread but specific areas of the brain. The most significant and consistent structural imaging findings include smaller total brain volumes, and reduced volumes in the right frontal lobe, right parietal cortex, caudate nucleus, cerebellar hemispheres, and posterior-inferior lobules of the cerebellar vermis. ADHD involves hypofunction of catecholaminergic circuits, particularly those that project to the prefrontal cortex. A minimum of 18 genes have been reported to be associated with the disorder; among them the DRD4 7-repeat allele has been found associated with a thinner prefrontal and posterior parietal cortex. Epigenetic factors acting during critical periods of prenatal and postnatal development may interact with genetic determinants. Methylphenidate, as well as the catecholaminergic nonstimulant atomoxetine, are effective in improving ADHD symptoms.