Spectrum of intracranial incidental findings on pediatric brain magnetic resonance imaging: What clinician should know?

Intracranial incidental findings on magnetic resonance imaging (MRI) of the brain continue to generate interest in healthy control, research, and clinical subjects. However, in clinical practice, the discovery of incidental findings acts as a "distractor". This review is based on existing heterogeneous reports, their clinical implications, and how the results of incidental findings influence clinical management. This draws attention to the followings: (1) the prevalence of clinically significant incidental findings is low; (2) there is a lack of a systematic approach to classification; and discusses (3) how to deal with the detected incidental findings based a proposed common clinical profile. Individualized neurological care requires an active discussion regarding the need for neuroimaging. Clinical significance of incidental findings should be decided based on lesion's neuroradiologic characteristics in the given clinical context. Available evidence suggests that the outcome of an incidentally found "serious lesion in children" is excellent. Future studies of intracranial incidental findings on pediatric brain MRI should be focused on a homogeneous population. The study should address this clinical knowledge based review powered by the statistical analyses.

Spectrum of migraine variants and beyond: The individual syndromes in children.

"Migraine-related conditions" are probably the second most common condition after seizure encountered in pediatric neurology requiring frequent Emergency Department visits. Among migraines, migraine-related condition presents with an acute onset sign or symptom other than headache or visual aura of unknown etiology. A delay in diagnosis is a common occurrence. Previously, the authors proposed a common clinical profile and suggested that the future review should seek the applicability of the common profile in aid to clinical diagnosis of migraine-related individual syndromes. Authors describe the clinical characteristics and differential diagnosis of the spectrum of migraine variants and beyond in children.

Spectrum of complicated migraine in children: A common profile in aid to clinical diagnosis.

Complicated migraine encompasses several individual clinical syndromes of migraine. Such a syndrome in children frequently presents with various neurological symptoms in the Emergency Department. An acute presentation in the absence of headache presents a diagnostic challenge. A delay in diagnosis and treatment may have medicolegal implication. To date, there are no reports of a common clinical profile proposed in making a clinical diagnosis for the complicated migraine. In this clinical review, we propose and describe: (1) A common clinical profile in aid to clinical diagnosis for spectrum of complicated migraine; (2) How it can be used in differentiating complicated migraine from migraine without aura, migraine with aura, and seizure; (3) We discuss the status of complicated migraine in the International Headache Society classification 2013; and (4) In addition, a common treatment strategy for the spectrum of migraine has been described. To diagnose complicated migraine clinically, it is imperative to adhere with the proposed profile. This will optimize the use of investigation and will also avoid a legal implication of delay in their management. The proposed common clinical profile is incongruent with the International Headache Society 2013. Future classification should minimize the dissociation from clinically encountered syndromes and coin a single word to address collectively this subtype of migraine with an acute presentation of a common clinical profile.

Intracranial hemorrhage in term newborns: management and outcomes.

Child neurology is frequently a late player in the management of the term newborn with intracranial hemorrhage in the first neonatal week. It is crucial, however, that the child neurologist undertake a comprehensive evaluation by investigating etiology and management of the hemorrhage. Intracranial hemorrhage is usually associated with premature newborns. The literature on intracranial hemorrhage in term newborns is largely in the form of isolated case reports or a small series of cases, and mostly nonsystematic. Presented here is an evidence-based review of the incidence, risk factors, etiologies, and clinical management of intracranial hemorrhage in the first week after birth, with discussion of the role of neuroimaging and hematologic investigation. Consideration of these investigations along with documentation of every intervention or its explanation will reduce parental anxiety and will assure the best possible neurologic as well as legal outcomes of term newborns with intracranial hemorrhage.

Skin-impedance in Fabry Disease: a prospective, controlled, non-randomized clinical study.

BACKGROUND: We previously demonstrated improved sweating after enzyme replacement therapy (ERT) in Fabry disease using the thermo-regularity sweat and quantitative sudomotor axon reflex tests. Skin-impedance, a measure skin-moisture (sweating), has been used in the clinical evaluation of burns and pressure ulcers using the portable dynamic dermal impedance monitor (DDIM) system. METHODS: We compared skin impedance measurements in hemizygous patients with Fabry disease (22 post 3-years of bi-weekly ERT and 5 ERT naive) and 22 healthy controls. Force compensated skin-moisture values were used for statistical analysis. Outcome measures included 1) moisture reading of the 100th repetitive reading, 2) rate of change, 3) average of 60-110th reading and 4) overall average of all readings. RESULTS: All outcome measures showed a significant difference in skin-moisture between Fabry patients and control subjects (p < 0.0001). There was no difference between Fabry patients on ERT and patients naïve to ERT. Increased skin-impedance values for the four skin-impedance outcome measures were found in a small number of dermatome test-sites two days post-enzyme infusions. CONCLUSION: The instrument portability, ease of its use, a relatively short time required for the assessment, and the fact that DDIM system was able to detect the difference in skin-moisture renders the instrument a useful clinical tool.

Parietal bone defect: differential diagnosis and neurologic associations.

Parietal bone defects are rare and exhibit variable etiologies. We report on a 16-year-old girl with an isolated, giant parietal bone defect with encephalomalacia, and an asymptomatic Rathke's cleft cyst. The patient presented with epilepsy. We discuss the differential diagnosis and pertinent neurologic associations. Irrespective of cause, parietal bone defects remain a benign clinical entity. However, it is important to define the extent of the bone defect and associated intracranial abnormalities, and if needed, to take early preventive steps, medical as well as surgical, against potential brain damage.

Intracranial incidental findings on brain MR images in a pediatric neurology practice: a retrospective study.

BACKGROUND AND PURPOSE: Previous studies have addressed the prevalence of incidental findings largely in healthy adult and pediatric populations. Our study aims to elucidate the prevalence of incidental findings in a pediatric neurology practice. METHODS: We reviewed the charts of 1618 patients seen at a pediatric neurology practice at a tertiary care center from September 2003 to December 2005 for clinical data and incidental intracranial findings on brain magnetic resonance imaging reports. Incidental findings were divided into two categories: normal or abnormal variants. Clinical and demographic data were assessed for associations with incidental findings. RESULTS: From 1618 charts reviewed, only 666 patients (41% of all patients) had brain MRIs ordered. One-hundred and seventy-one (171) patients (25.7% of all patients; 95% CI: 22.6, 29.0) had incidental findings. Of these, 113 (17.0%; 95% CI: 14.1, 19.8) were classified as normal-variants and 58 (8.7%; 95% CI: 6.6, 10.9) were classified as abnormal. The nature of incidental findings was not related to age group, sex or clinical diagnosis (p=0.29, p=0.31 and p=0.69 respectively). Two patients (0.3%; 95% CI: approximately 0.0, 0.7) required neurosurgical referral. CONCLUSIONS: We report a high prevalence of and a low rate of referrals for incidental findings in comparison to previous studies. The present study may help guide management decisions and discussions with patients and families. Future studies should attempt to address issues of associations between primary or secondary diagnoses and intracranial incidental findings in a controlled, prospective fashion.

Spinocerebellar ataxia type 7 mimicking Kearns-Sayre syndrome: a clinical diagnosis is desirable.

Spinocerebellar ataxias are a group of autosomal dominant cerebellar degenerative disorders, which are characterized by clinical and genetic variability. Spinocerebellar ataxia type 7 (SCA7) is less variable in clinical presentation than other SCAs. We present a pediatric patient with 13 and 70 trinucleotide CAG repeats within SCA7 gene and no family history, whose presentation mimicked Kearns-Sayre syndrome (KSS). We review the differential diagnosis of cerebellar ataxia with vision loss secondary to retinal pigmentary dystrophy. This paper supports concept of a desirable clinical diagnosis to avoid multiple genetic or invasive testing in children with neurodegenerative disorders.