Mossy and climbing fibre organization on the anterior lobe of the cerebellum activated by forelimb and hindlimb areas of the sensorimotor cortex

Summary Stimulation of forelimb and hindlimb areas of the sensorimotor cortex in the cat evokes in the lobus anterior of the cerebellum an early response at latency of 3–3.5 msec due to the mossy fibre input (MF) and a later response at latency of 13–16 msec due to the climbing fibre (CF) input.In the pars intermedia these two types of responses are organized in a somatotopic manner: the hindlimb area projects in lobuli HIV and HIII whereas the forelimb area projects to lobulus HV. In the vermis a somatotopic arrangement is less clear. Both forelimb and hindlimb areas of the sensorimotor cortex project to lobuli III, IV and V: on a maintained somatotopy in a caudo-rostral direction there is a tendency for the hindlimb area of the sensorimotor cortex to be well represented in a longitudinal strip close to the paravermal sulcus. This arrangement in the vermis is evident for the CF pathways, but more difficult to demonstrate for the MF pathways.The forelimb area of the sensorimotor cortex projects to those areas of the lobus anterior impinged upon by the forelimb nerves through both the MF and CF systems and the same holds true for the hindlimb area and the hindlimb nerves.     

The wide clinical spectrum of nocturnal frontal lobe epilepsy

Nocturnal frontal lobe epilepsy (NFLE) has become clinically relevant in recent years. NFLE represents a spectrum of clinical manifestations, ranging from brief, stereotyped, sudden arousals, often recurring several times per night, sometimes with a quasi-periodic pattern, to more complex dystonic-dyskinetic seizures and to prolonged "somnambulic" behaviour. Episodes of increasing intensity have been labelled as paroxysmal arousal (PA), nocturnal paroxysmal dystonia (NPD) and episodic nocturnal wandering (ENW). NFLE affects both sexes with a higher prevalence for men, is frequently cryptogenetic and displays a strong familial trait for parasomnias and epilepsy (NFLE). Seizures appear more frequently between 14 and 20 years of age, but can affect any age and tend to increase in frequency during life. Interictal and ictal scalp electroencephalography (EEG) are often normal, the use of sphenoidal leads may be helpful. Carbamazepine taken at night is often effective at low doses, but a third of the patients are resistant to anti-epileptic drugs (AED) treatment. A familial form, characterized by an autosomal dominant transmission, has also been described. Autosomal dominant nocturnal frontal lobe epilepsy is a genetic variant of NFLE, in itself both clinically and biologically heterogeneous. NFLE should be suspected in the presence of frequent stereotyped paroxysmal nocturnal motor events arising or persisting into adulthood. Videopolysomnography is mandatory to confirm the diagnosis.     

Mutations in mammalian target of rapamycin regulator DEPDC5 cause focal epilepsy with brain malformations

We recently identified DEPDC5 as the gene for familial focal epilepsy with variable foci and found mutations in >10% of small families with nonlesional focal epilepsy. Here we show that DEPDC5 mutations are associated with both lesional and nonlesional epilepsies, even within the same family. DEPDC5‐associated malformations include bottom‐of‐the‐sulcus dysplasia (3 members from 2 families), and focal band heterotopia (1 individual). DEPDC5 negatively regulates the mammalian target of rapamycin (mTOR) pathway, which plays a key role in cell growth. The clinicoradiological phenotypes associated with DEPDC5 mutations share features with the archetypal mTORopathy, tuberous sclerosis, raising the possibility of therapies targeted to this pathway. Ann Neurol 2014;75:782–787     

Toxicity of 4-nonylphenol to Tubifex tubifex and Chironomus riparius in 28-day whole-sediment tests

Concern has been growing recently over the toxicity and the mimicking properties of 4-nonylphenol (4NP), the most persistent breakdown product of the nonionic surfactant nonylphenol ethoxylates, broadly used in industrial cleaning processes. Twenty-eight-day tests were performed to evaluate the toxicity and the effects on reproduction of 4NP spiked sediment to the benthic invertebrates Tubifex tubifex and Chironomus riparius. 4NP was directly added to the wet sediment, avoiding any solvent. The EC50 value for the emergence of chironomids was 380 microg 4NP g(-1) dry weight (dw); the EC50 values for the deposition of cocoons and the development of young worms were very similar (428 and 420 microg g(-1) dw, respectively). Regarding NP estrogenic activity and its effects on reproduction and development, no significant differences were noted in the sex ratio of the emerged chironomids when exposed to 4NP compared to the controls, but the emerged chironomids did not lay eggs at concentrations higher than the EC10 (250 microg 4NP g(-1) dw). Furthermore, at concentration of 610 microg 4NP g(-1) dw an interference in the development of the gonads of adult worms was observed, but it was impossible to determine whether this effect was due to 4NP toxicity or to its hormone mimicking properties.     

Bilateral paramedian thalamic syndrome: abnormal circadian wake-sleep and autonomic functions

OBJECTIVES: To describe wake-sleep and body core temperature (t degrees ) rhythm abnormalities in two patients with bilateral paramedian thalamic calcifications. METHODS: Patients underwent (18F)FDG PET scans and 24 hour polygraphic recordings of wake-sleep and t degrees. RESULTS: PET showed bilateral thalamic hypometabolism in both patients with additional basal ganglia or mesiolateral frontal and cingular hypometabolism. Wake-sleep studies showed abnormal sleep organisation and in the case with frontal and limbic PET hypometabolism, pre-sleep behaviour associated with "subwakefulness" EEG activities, lack of EEG spindles and K complexes, and features of status dissociatus. The t degrees rhythms showed increased mesor in both (37.4 degrees C and 37.75 degrees C) and inverted rhythm in one patient. CONCLUSIONS: Paramedian thalamic structures and interconnected, especially frontal and cingular, areas play a part in the organisation of the wake-sleep cycle and attendant autonomic functions.     

Eruptive pseudoangiomatosis

Eruptive pseudoangiomatosis is a rare, benign, spontaneously regressive disease. The term was recently coined to describe a dermatosis characterized by the sudden onset of a few to several bright red angioma-like papules with histopathologic findings distinct from that of true angiomas. We describe a 7-year-old patient with the typical lesions of eruptive pseudoangiomatosis.     

Partial Epilepsy of Long Duration: Changing Semiology with Age

There are few data on changing patterns of localization-related epileptic syndromes with time and particularly on changes in seizure semiology as patients age. We retrospectively reviewed 53 patients aged >60 years who had had partial epileptic seizures for a mean duration of 44 years. In 20 patients, seizures became progressively less elaborate and briefer with time. In 29 patients, seizures were unchanged. In four patients, seizures worsened, with the appearance of drop attacks, secondarily generalized seizures, increasing drug resistance, and mental deterioration. These latter 4 patients had cerebrovascular disease with multiple strokes.     

Insomnia in neurological diseases

Insomnia is the most common sleep complaint. Insomnia is not a disease itself but mostly a clinical sign of an underlying disease. Degenerative and vascular diseases involving the central nervous system (CNS) may impair sleep either as a result of the brain lesion or because of illness-related discomfort (motor immobility, social and familial impairment, depression, drugs). Some neurological conditions characterized by movement disorders that start or persist during sleep hinder sleep onset and/or sleep continuity, causing a poor sleep complaint. CNS lesions and/or dysfunction in three specific neurological conditions (fatal familial insomnia, Morvan's chorea, and delirium tremens) impair the basic mechanisms of sleep generation inducing a syndrome in which the inability to sleep is consistently associated with motor and sympathergic overactivation. Agrypnia excitata is the term that aptly defines this generalized overactivation syndrome.     

Risk for human health of some POPs due to fish from Lake Iseo

Risk evaluation for human health was carried out using PCB and PAH concentrations measured in seven fish species from Lake Iseo (Italy) and comparing the experimental data with legal levels and the two new approaches proposed by the US FDA and the US EPA to protect local populations with different food habits. We verified that none of the persistent organic compounds analyzed exceeded the legal limits set by the US FDA, since no specific limit has yet been proposed by the European Union. The two other approaches have pointed out that the acute and chronic effects do not represent any risk for human health, while a possible carcinogenic risk for the local population can arise when 4 and 8 monthly meals are consumed, based on PCB and PAH content in the edible part of fish, respectively.