Idiopathic hypereosinophilic syndrome (HES) is a heterogeneous disorder characterized by prolonged eosinophilia without an identifiable cause, ultimately resulting in organ dysfunction. Three major types of neurologic involvement have been well defined in HES; however, to our knowledge, inflammatory pseudotumor (IPT) in association with HES has not been reported. We present a case of IPT of the...

Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) is a rare multisystemic autosomal recessive disorder characterized by ptosis, gastrointestinal dysmotility, cachexia, peripheral neuropathy, and leukoencephalopathy. We aimed to raise awareness in radiologists regarding this difficult-to-diagnose syndrome, which occurs in the presence of coexistent gastrointestinal dysmotility, cache...

Hashimoto’s encephalopathy (HE) is a rare disorder first characterized by Brain et al. in 1966 [1]. With only a few hundred reported cases, its etiology is not entirely understood but is believed to be related to the autoimmune mechanism of Hashimoto’s thyroiditis. Generally, patients diagnosed with HE demonstrate a progressive encephalopathy in the setting of elevated antithyroid antibodies. T...

Gri:inblad-Strandberg syndrome known as pseudoxanthoma elasticum (PXE) is a rare connective tissue disorder, the inheritance of which shows both autosomal and recessive characteristics [1]. The disease most commonly involves the skin , eyes , cardiovascular system, and gastrointestinal tract [2]. The vascular connective tissue supporting the retina, known as the Bruch membrane, tends to calcify...

Seizures are a major neurologic disorder in infants and children. It can affect children at any age, from birth through adolescence. Early onset of seizures or the first few months life cause severe cerebral dysfunction. Also, recurrent persistent lead to cognitive behavioral impairment Hence, control is crucial for optimal neurodevelopment. Genetic aetiology suspected early below one year refr...

Neurologic disease is believed to be an unusual complication during the course of chronic lymphocytic leukemia. Nevertheless, it has already been proven in autopsy series that the incidence of occult nervous system infiltration is much higher than was previously expected. The advent of more potent drugs to treat this lymphoproliferative disorder has brought a new hope for a possible cure in the...

Serial MR imaging and quantitative proton MR spectroscopic imaging (MRSI) findings of a 4-year-old boy with acute disseminated encephalomyelitis (ADEM) are reported. Over a 2-month period characterized by an initial illness and two relapses, each with full recovery, MR imaging exhibited the appearance and disappearance of multifocal lesions throughout the CNS that correlated only partly with th...

Background: The family of disorders associated with antibodies against neuronal surface antigens is a rapidly expanding category of autoimmune neurologic disease. Methods: Clinical and laboratory findings of a patient with voltage-gated Kv1 potassium channel (VGKC)-complex autoantibodies are reported and the literature is reviewed. Results: New-onset faciobrachial dystonic seizures, distal larg...

Tumor cells can express so-called onconeural antigens, which are normally restricted to mature neurons and glial cells in the CNS. The detection of neuralreactive immunoglobulin G (IgG) aids the diagnosis of paraneoplastic neurologic syndromes (PNS); however, the diagnostic utility and potential pathogenicity of autoantibodies vary between neurologic diseases. By contrast, anti-aquaporin-4 (AQP...