Tumour type and size are high risk factors for the syndrome of "cerebellar" mutism and subsequent dysarthria.
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OBJECTIVE: "Cerebellar mutis" and subsequent dysarthria (MSD) is a documented complication of posterior fossa surgery in children. In this prospective study the following risk factors for MSD were assessed: type, size and site of the tumour; hydrocephalus at presentation and after surgery, cerebellar incision site, postoperative infection, and cerebellar swelling. METHODS: In a consecutive series of 42 children with a cerebellar tumour, speech and neuroradiological studies (CT and MRI) were systematically analysed preoperatively and postoperatively. Speech was assessed using the Mayo Clinic lists and the severity of dysarthria using the Michigan rating scale. RESULTS: Twelve children (29%) developed MSD postoperatively. The type of tumour, midline localisation, and vermal incision were significant single independent risk factors. In addition, an interdependency of possible risk factors (tumour>5 cm, medulloblastoma) was found. CONCLUSION: MSD often occurs after paediatric cerebellar tumour removal and is most likely after removal of a medulloblastoma with a maximum lesion diameter>5 cm. (+info)
The cerebellum contributes to higher functions during development: evidence from a series of children surgically treated for posterior fossa tumours.
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We present data on the intellectual, language and executive functions of 26 children who had undergone surgery for the removal of cerebellar hemisphere or vermal tumours. The children with right cerebellar tumours presented with disturbances of auditory sequential memory and language processing, whereas those with left cerebellar tumours showed deficits on tests of spatial and visual sequential memory. The vermal lesions led to two profiles: (i) post-surgical mutism, which evolved into speech disorders or language disturbances similar to agrammatism; and (ii) behavioural disturbances ranging from irritability to behaviours reminiscent of autism. These data are consistent with the recently acknowledged role of the cerebellum as a modulator of mental and social functions, and suggest that this role is operative early in childhood. (+info)
Selective mutism and obsessive compulsive disorders associated with zonisamide.
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We treated 27 children with idiopathic epilepsy with zonisamide monotherapy over a period of 2 years and observed behaviour disturbances in a prospective study. In all cases, seizure control was excellent; however, two cases (7.4%) had behaviour disturbances. The first (Case 1) was a 14-year-old girl with partial epilepsy which began at age 4 years. Zonisamide was administered at age 6 years, which was effective against her seizures, but selective mutism, violent behaviour, and lack of concentration developed at age 10 years. The second (Case 2) was a 15-year-old girl with generalized tonic-clonic seizures which began at age 10 years. Zonisamide was also effective against her seizures, but obsessive compulsive disorders (OCD) developed at age 13 years. The patients have had no other physical or mental problems and decreasing the dosage of zonisamide reduced the problems. There are few reports of behaviour disturbances provoked by zonisamide monotherapy in epileptic children who are neither physically nor mentally disturbed. While problems can develop several years later, in the present study, decreasing the zonisamide dosage maintained adequate prevention of seizures and eliminated the behaviour disturbances. Zonisamide is still a useful anticonvulsant for epileptic seizures, but physicians should be wary of its adverse behavioural side effects, which may arise several years later. (+info)
Neglected child with substance abuse leading to child abuse: a case report.
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Child abuse and neglect is any interaction or lack of interaction between a caregiver and a child resulting in nonaccidental harm to the child's physical and developmental state. Substance abuse is ingestion of any drug, which is capable of altering the mental functioning eventually leading to addiction. This paper presents a case report of a 12-year-old neglected girl with substance abuse for which she was physically abused by her mother. (+info)
Contributions of intrinsic membrane dynamics to fast network oscillations with irregular neuronal discharges.
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During fast oscillations in the local field potential (40-100 Hz gamma, 100-200 Hz sharp-wave ripples) single cortical neurons typically fire irregularly at rates that are much lower than the oscillation frequency. Recent computational studies have provided a mathematical description of such fast oscillations, using the leaky integrate-and-fire (LIF) neuron model. Here, we extend this theoretical framework to populations of more realistic Hodgkin-Huxley-type conductance-based neurons. In a noisy network of GABAergic neurons that are connected randomly and sparsely by chemical synapses, coherent oscillations emerge with a frequency that depends sensitively on the single cell's membrane dynamics. The population frequency can be predicted analytically from the synaptic time constants and the preferred phase of discharge during the oscillatory cycle of a single cell subjected to noisy sinusoidal input. The latter depends significantly on the single cell's membrane properties and can be understood in the context of the simplified exponential integrate-and-fire (EIF) neuron. We find that 200-Hz oscillations can be generated, provided the effective input conductance of single cells is large, so that the single neuron's phase shift is sufficiently small. In a two-population network of excitatory pyramidal cells and inhibitory neurons, recurrent excitation can either decrease or increase the population rhythmic frequency, depending on whether in a neuron the excitatory synaptic current follows or precedes the inhibitory synaptic current in an oscillatory cycle. Detailed single-cell properties have a substantial impact on population oscillations, even though rhythmicity does not originate from pacemaker neurons and is an emergent network phenomenon. (+info)
Waardenburg syndrome type 1.
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Waardenburg syndrome (WS) is a rare disease characterized by sensorineural deafness in association with pigmentary anomalies and defects of neural-crest-derived tissues. Depending on additional symptoms, WS is classified into four types, WS1, WS2, WS3 and WS4. WS1 and WS3 are attributed to mutations in PAX3, whereas WS2 is heterogeneous, being caused by mutations in the microphthalmia-associated transcription factor gene in some but not all affected families. WS4 is attributed to mutations in the endothelin-3 or the endothelin-B receptor genes and SOX10 gene. WS1 is an auditory-pigmentary disorder comprising sensorineural hearing loss and pigmentary disturbances of the iris, hair, and skin, along with dystopia canthorum. We report a case with a confirmed diagnosis of WS1 and review the relevant literature for this rare disorder. (+info)
Cerebellar mutism following posterior fossa tumor resection in children.
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AIM: Cerebellar mutism is a documented complication of posterior fossa surgery in pediatric ages. Risk factors such as the type of tumor, size, and location of tumor, hyrdrocephalus, postoperative cerebellar swelling for cerebellar mutism were investigated in this study. MATERIAL AND METHODS: A consecutive series of 32 children with a cerebellar tumor were operated on at the Haseki Educational and Research Hospital, Department of Neurosurgery, between 1990 and 2005. Their speech and neuroradiological studies were systematically analysed both preoperatively and postoperatively RESULTS: Cerebellar mutism developed in ten children (32%) in the early postoperative period. The type of tumor, midline localization, and vermian incision were significant single independent risk factors. In addition, an interdependency of possible risk factors (tumor > 5 cm, medulloblastoma) was found. The latency for the development of mutism ranged from 0 to 90 days (mean 15.6 d). The speech returned to normal in eight patients. All cases were accompanied by cerebellar ataxia. CONCLUSION: Mutism after posterior fossa tumor resection is also associated with ataxia. Cerebellar mutism usually has a self-limiting course and a favorable prognosis. (+info)
Parental responses to positive and negative emotions in anxious and nonanxious children.
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