Multisystem inflammatory syndrome in neonates associated with SARS-CoV-2 infection, a different entity?

BACKGROUND: Multisystemic inflammatory syndrome in children (MIS-C) is a novel disease that is associated with the severe acute respiratory syndrome coronavirus 2 (SARS-CoV2). MIS-C usually affects children older than 5 years of age and adolescents, with a median of 8-years and an interquartile range of 3 to 11 years. A multisystemic inflammatory disease has been described in neonates and named MIS-N (multisystemic inflammatory syndrome in Neonates). We report three cases of Mexican newborns with MIS-N presenting with multiorgan compromise and a positive anti-SARS-CoV-2 IgG who developed Kawasaki disease (KD)-like cardiac features and discuss the current dilemma regarding diagnosis and treatment in these patients.

[An atypical progression of rolandic epilepsy: the value of single-photon emission computerised tomography co-registered to magnetic resonance imaging]. / Evolución atípica de la epilepsia rolándica: aportaciones de la tomografía computarizada por emisión de fotón único corregistrada con imágenes de resonancia magnética.

INTRODUCTION: The complicated forms of rolandic epilepsy progress with a continuous spike-wave pattern in slow-wave sleep. Experiments conducted in cats suggest that this pattern can only appear if there is bilateral thalamic insult. AIM. To determine whether thalamic hypoperfusion is associated with the complicated variants of rolandic epilepsy. PATIENTS AND METHODS: A group of 24 children were studied over a period of six years following their first epileptic seizure. During the follow-up an interictal magnetic resonance scan and single-photon emission computerised tomography (SPECT) were performed. Results were examined to ascertain whether there were asymmetries in the distribution of cerebral blood flow through structures, using parametric statistical maps. The brain SPECT was performed when progression to atypical benign partial epilepsy in infancy was diagnosed and in typical forms of rolandic epilepsy when there was some mild neuropsychological deficit that led the specialist to suspect the existence of a focal cortical lesion. RESULTS: Bilateral thalamic hypoperfusion was found in all patients diagnosed with atypical benign partial epilepsy in infancy, which was correlated with the presence of continuous spike-waves during the slow-wave phase of non-REM sleep. CONCLUSIONS: Bilateral thalamic hypoperfusion seems to be a necessary condition for the atypical progression of rolandic epilepsy.

[A prospective, open, controlled and randomised study of clobazam versus carbamazepine in patients with frequent episodes of Rolandic epilepsy]. / Estudio prospectivo, abierto, controlado y aleatorizado de clobazam frente a carbamacepina en pacientes con crisis frecuentes de epilepsia rolándica.

INTRODUCTION: To date no controlled, randomised studies with flexible dose regimens have been conducted in children with rolandic epilepsy, and therapy is therefore still empirical. AIM: To evaluate the effectiveness and safety of clobazam (CLB) compared with that of carbamazepine (CBZ) in rolandic epilepsy. PATIENTS AND METHODS: A prospective, open, controlled and randomised study was carried out to compare CBZ and CLB in children with rolandic epilepsy with a follow-up over a two-year period. A random sample of 45 patients was taken and 38 of them finished the study. A flexible dose regimen was indicated. Control of seizures, academic performance, behaviour, adherence to treatment, parents' degree of satisfaction and side effect profiles were all evaluated. RESULTS: Both drugs were equally effective at controlling seizures (94.1% of patients with CLB and 100% of those with CBZ were free of seizures on ending the study; p = 0.26). CLB controlled seizures earlier (33.3 +/- 45 days versus 48.2 +/- 72.3; p < 0.05) and had fewer side effects than CBZ (side effects appeared in three patients with CLB and in eight of those on CBZ; p = 0). In two of the patients taking CBZ, the seizures got worse and a series of cognitive-behavioural complications also appeared. CONCLUSIONS: CBZ is an effective drug in rolandic epilepsy, but it may be associated with exacerbation of seizures as well as with cognitive-behavioural impairment. CLB in monotherapy seems to be an effective and better tolerated drug in this kind of epilepsy.

[Unilateral neglect, transient cognitive impairment and intercritical activity in Rolandic epilepsy]. / Heminegligencia, déficit cognitivo transitorio y actividad intercrítica en la epilepsia rolándica.

INTRODUCTION: Benign focal epilepsy in infancy with centro-temporal paroxysms is a frequent form of epilepsy within this group of epilepsies. Despite its relative benignity, however, it may be accompanied by neuropsychological deficits and therefore constitutes a suitable in vivo model for studying how the brain functions when processing information. CASE REPORT: We report the case of a 7-year-old child who began with this type of epilepsy by manifesting focal seizures during the early stages of sleep and who, with the absence of any continuous spike-wave activity in non-REM sleep, presented transient unilateral neglect syndrome on the right-hand side related with electroencephalographic intercritical activity. CONCLUSIONS: The neuropsychological manifestations in this type of epilepsy can be due to intercritical paroxysmal activity. The clinical features depend on where the paroxysms are located and in which direction they spread. A dysfunction of the physiological neuronal synchrony among the neuronal networks that are necessary for thinking processes could be the cause of this disorder.

[Neuropathies due to vasculitis in infancy]. / Neuropatias por vasculitis en la infancia.

INTRODUCTION: Vasculitis are characterised by the inflammatory infiltrate, chiefly of mononuclear cells, in the walls of the blood vessels, which can lead to occlusion with necrosis and the subsequent infarction of the affected tissue. AIMS: The Objective of this study was to determine the clinical, anatomicopathological and neurophysiological aspects of vasculitic neuropathies in infancy. PATIENTS AND METHODS: Each patient was submitted to the following tests: a complete hemogram, systemic lupus erythematosus cells (LE cells), lupus anticoagulant, antinuclear antibodies, neutrophil anticytoplasmic antibodies, venereal disease research laboratory test (VDRL), erythrocyte sedimentation rate, liver transaminases, serological testing for hepatitis C, B and A, cytochemical study of the cerebrospinal fluid, study of motor and sensory conduction, electromyography and nuclear magnetic resonance, when required; a biopsy of the sural nerve was performed, which was replaced by a necropsy if the patient died. RESULTS: 15 patients from a total of 25 who were studied had vasculitic neuropathies; the most usual presentation was multiple mononeuropathy; aetiologies found included microscopic polyangiitis, systemic lupus erythematosus, JRA, overlap syndrome and several undetermined vasculitis. Of the patients who were submitted to a biopsy, 75% showed signs of vasculitis, which affected the small and medium sized vessels in 62.5% of patients. CONCLUSIONS: The presence of neuropathy in association with symptoms and signs of systemic involvement suggested the possibility of a neuropathy in the course of a vasculitis. The examination of biopsy specimens of the sural nerve is useful for the diagnosis and classification of the aetiology of vasculitis. Response to treatment with immunosuppressant drugs was good, both in the case of the neuropathy and of the underlying disease.

[Recurring ischemic cerebral infarction caused by primary thrombocytosis in infancy]. / Infarto cerebral isquémico recurrente en la infancia por trombocitosis primaria.

INTRODUCTION: Ischemic cerebrovascular disease includes a set of entities that are produced by disorders in components of the blood, the blood flow, the walls of blood vessels or the heart, and can be anatomical, functional or even mixed. CASE REPORT: We describe the case of an 18-month-old male patient with compensated celiac disease, with repeated ischemic strokes in different territories, including the right posterior cerebral artery and middle cerebral artery, in the course of a hypercoagulable state due to essential thrombocytosis. Computerised axial tomography scans, brain angioresonance, a complete blood chemistry analysis and bone marrow biopsy were all performed and confirmed the previous diagnosis. Exchange transfusion, antiplatelet drugs and a platelet production inhibitor (anagrelide) were begun as therapy. At present, the patient is 2 years old and still has a slight direct hemiparesis, which is complete and predominantly faciobrachial, with no alterations to language. CONCLUSIONS: Cerebral infarctions in infancy are infrequent, and their presentation obliges the attending clinician to seek causes that are not usual. In our patient the hypocoagulability came about due to essential thrombosis, which is rare in infancy. The cause behind the infarction determines the chances of its recurring. Acetylsalicylic acid did not prove to be effective for this purpose. We suggest using carbamazepine for the treatment of kinesigenic dystonias.

[Multifocal motor neuropathy with partial block of nerve conduction]. / Neuropatía motora multifocal con bloqueo parcial de la conducción nerviosa.

INTRODUCTION: Multifocal motor neuropathy with partial conduction block is characterized by being a chronic, demyelinating, autoimmune severely disabling neuropathy. In Cuba three cases were reported by Dr. Estrada et al in 1999. This neuropathy presents clinically in relatively young persons. The arms are predominantly affected and the typical signs are of severe asymmetrical weakness, with atrophy which is less marked than the weakness, fasciculations, cramps and myokymiae of the affected muscle. Neurophysiological study shows partial block of motor nerve conduction. Clinical interest is due to it being potentially curable. Many cases are wrongly diagnosed as motor neurone disease. CLINICAL CASES: We present five patients aged under 55 years with progressive chronic motor neuropathy mainly affecting their arms. Study of nerve conduction showed partial block of the conduction in motor nerves, in segments with no block of sensory neuroconduction. One patient had been diagnosed as having motor neurone disease; another had slight sensory involvement in the distal territory of the radial nerve; in two patients the symptoms affected all four limbs. In three patients good results were obtained with intacglobin, followed by azothroprine and prednisone. Two patients showed no improvement with this treatment so intravenous cyclophosphamide was given for nine months which stopped progression of the disorder. CONCLUSIONS: Multifocal motor neuropathy is potentially treatable. In some cases intacglobin azathioprine and prednisone may be a useful alternative to cyclophosphamide.

[Opsoclonic-myoclonic-ataxic syndrome]. / Síndrome opsoclono-mioclono-atáxico.

INTRODUCTION: The opsoclonic-myoclonic-ataxic syndrome (OMAS) is that in which there are at least two of the cardinal signs (opsoclonus, myoclonus and ataxia). OBJECTIVES: To report three cases, their evolution and aetiology, and discuss the main physiopathological basis and guidelines for treatment. CLINICAL CASES: We present three cases admitted to the Internal Medicine Department with the diagnosis of OMAS. The investigations included standard biochemical and haematological tests, a full study of the cerebrospinal fluid, computerized axial tomography of the skull and thorax, and magnetic resonance. We describe the clinical presentation, evolution, aetiology and treatment given. The results of the complementary tests are shown in the form of tables. Finally we consider the diagnosis of this condition. Three patients, two men and one woman, aged between 43 and 78 were diagnosed as having OMAS. The causes were: paraneoplasia, acute diffuse encephalomyelitis and probable multiple sclerosis. The patient with lung cancer died whilst the other two showed almost complete recovery on the treatment given. CONCLUSIONS: Our cases show that OMAS may be a feature of a diffuse process affecting the brainstem and cerebellum. The commonest causes found are multiple sclerosis, acute diffuse encephalomyelitis and as a paraneoplastic feature of a tumour. It may improve with treatment, but depending on the aetiology, death may occur. Immunomodulation treatment is vital even though symptomatic treatment may be given.