Spina Bifida Occulta

This common anomaly consists of a midline defect of the vertebral bodies without protrusion of the spinal cord or meninges. Most individuals are asymptomatic and lack neurologic signs, and the condition is usually of no consequence. In some cases, patches of hair, a lipoma, discoloration of the skin, or a dermal sinus in the midline of the lower back suggests a more significant malformation of the spinal cord .

A spine roentgenogram in simple spina bifida occulta shows a defect in closure of the posterior vertebral arches and laminae, typically involving L5 and S1; there is no abnormality of the meninges, spinal cord, or nerve roots. Spina bifida occulta is occasionally associated with more significant developmental abnormalities of the spinal cord, including syringomyelia, diastematomyelia, and a tethered cord. These are best identified with MRI . Some consider the term spina bifida occulta to denote merely a posterior vertebral body fusion defect. This simple defect does not have an associated spinal cord malformation.
 Other clinically more significant forms are more correctly termed occult spinal dysraphism.
In most of these cases, there are cutaneous manifestations such as a hemangioma, pit, lump, or hairy patch .
A dermoid sinus usually forms a small skin opening, which leads into a narrow duct, sometimes indicated by protruding hairs, a hairy patch, or a vascular nevus. Dermoid sinuses occur in the midline at the site of where meningoceles or encephaloceles may occur: the lumbosacral region or occiput. Dermoid sinus tracts may pass through the dura, acting as a conduit for the spread of infection. Recurrent meningitis of occult origin should prompt careful examination for a small sinus tract in the posterior midline region, including the back of the head. Lower back sinuses are usually above the gluteal fold and are directed cephalad. Tethered spinal cord syndrome may also be an associated problem.

Multiple Sclerosis

MS is a chronic and generally remitting-relapsing disorder characterized by multiple white lesions in the CNS separated by time and location in the brain. The condition is rare in the pediatric population; 5% of all cases of MS occur before age 18 yrEtiology:

The cause of MS is unknown, but interactive genetic, immunologic, and infectious factors are probably responsible. A family history of MS in a first-degree relative is present in ?20%

Clinical Features:

The most frequent presenting signs are unilateral weakness with upper motor neuron signs, sensory abnormalities, visual complaints, or ataxia. Paresthesias involving the lower extremities, distal portions of the hands and feet, and the face are common.

Visual symptoms including diplopia, nystagmus, or sudden visual loss due to optic neuritis are also important early manifestations of MS.

Headache, fatigue, dysarthria, or myelopathy with a sensory level and neurogenic bladder can also be present, but impaired consciousness and encephalopathy are uncommon

Pathology:

The pathology of MS consists of demyelination with the formation of plaques

Diagnosis:

No reliable laboratory test unequivocally confirms the diagnosis of MS, except for a biopsy or an autopsy. MRI is the neuroimaging technique of choice; small plaques of 3–4 mm can be identified, particularly those located in the brainstem and spinal cord

A high percentage of pediatric patients have T2 enhancing lesions in the corpus callosum and periventricular white matter. The cerebrospinal fluid (CSF) often contains oligoclonal bands

Etiology of Pseudotumor cerebri

There are many explanations for the development of pseudotumor cerebri, including alterations in CSF absorption and production, cerebral edema, abnormalities in vasomotor control and cerebral blood flow, and venous obstruction.

The causes of pseudotumor are numerous and include

metabolic disorders (galactosemia, hypoparathyroidism, pseudohypoparathyroidism, hypophosphatasia,

prolonged corticosteroid therapy or too rapid corticosteroid withdrawal,

possibly growth hormone treatment,

refeeding of a significantly malnourished child,

hypervitaminosis A,

vitamin A deficiency,

Addison disease,

obesity,

menarche,

oral contraceptives, and pregnancy),

infections (roseola infantum, sinusitis, chronic otitis media and mastoiditis, Guillain-Barré syndrome),

drugs (nalidixic acid, doxycycline, minocycline, tetracycline, nitrofurantoin),

isotretinoin used for acne therapy especially when combined with tetracycline,

hematologic disorders (polycythemia, hemolytic and iron-deficiency anemias, Wiskott-Aldrich syndrome),

obstruction of intracranial drainage by venous thrombosis (lateral sinus or posterior sagittal sinus thrombosis),

head injury, and

obstruction of the superior vena cava.

When a secondary cause is not identified, the condition is classified as “idiopathic intracranial hypertension.”