HYDROCEPHALUS (H) & SPINA BIFIDA (SP BI) SAM LAZUARDI

SPINA BIFIDA (SP BI)Spina bifida (split spine) : incomplete closure of embryonic neural tube, overlying vertebrae un-fused & open abn portion of sp cord stick out through opening in bonesSp bi 4 categories : sp bi occulta, sp bi cystica (myelomeningocele), meningocele, lipo-meningoceleLocation : lumbar & sacralMyelomeningocele disability

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SPINA BIFIDA (SP BI)Sp bi can be closed surgically, but damage is permanentSp bi incidence can be 70 %, by daily folic acid intake before conceptionSp bi lumbar symptoms hip, leg, knee, foot muscles & scoliosisSacral foot muscles, bowel- bladder problems, erectionOften hydrocephalus, rarely intellectual problems*

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SPINA BIFIDA (SP BI)Tethered sp cord sy (TSC) as a result from scar tissue following surgery to close open defect sp bi myelomeningocele early D/ & Th / before permanent damage TSC symptoms : lower body pain, leg weakness, incontinence, scoliosis, numbness73 % Sp bi allergy to latex (gloves, catheter etc)*

SPINA BIFIDA (SP BI)Sp bi occulta (hidden) : no opening, but outer part of vertebra not completely closed skin normal / some hair growing, dimple in skin, lipoma or dermal sinus, birthmarkMay suffer TSC sy from diastematomyelia or lipoma in lipomyelomeningocele sp cord trapped below affected level of growing spine legs & bladder symptomsBack pain worse with sp bi occulta

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SPINA BIFIDA (SP BI)Meningocele or meningeal cyst (least common) Sp bi cystica most common & seriousUnfused portion of sp collumn allows sp cord protrude through opening in overlying vertebrMeningeal membrane covering sp cord form a sac enclosing spinal elementsSp bi + myeloschizis most severe : plate-like mass of nervous tissue with no overlying skin prone to infections

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SPINA BIFIDA (SP BI)Depending of locations deformities of hip knees, feet loss of muscle tone, some degree of paralysis, loss of sensation below level of spinal defect, bladder bowel control problemsAssociated abnormalities : Arnold chiari II (cerebellum malformation), myelome-ningocele, hydrocephalus*

SPINA BIFIDA (SP BI)Pathophysiology :neural tube closure 28 days postfertilizationCause : multiple gene & environmental factors (anticonvulsant, antidiabetic drugs)Women with risk factor high dose folic acid (4 mgr/d) 3 mo before conception - 12 wks during pregnancy (normal 0.4 mgr / d)*

SPINA BIFIDA (SP BI)Folic acid in leaf veggies, whole grains, fruits & beansPregnancy screening : AFP(alfa fetopro- tein for Down sy, SP BI, Neural Tube Defect), USG, amniocentesis, genetic counseling.Incidence 1 2 per 1000 birthsTreatment : surgery (sp cord & nerve roots put back into spinal canal & covered with meninges) & shunt for hydrocephalus*

OBESE WOMENObese women significantly more likely to have children with birth defect such as spina bifida, heart defects, cleft lip than slim mothersAn estimated one in six pregnant women in Britain is overweight (10 Febr 2009)*

Vitamin B12 Deficiency Can Lead To Childhood Brain Damage Women with low levels of Vitamin B12 just before or after conception are more likely to give birth to children with neural tube defects (Pediatrics, February 2009)The study authors wrote that it is not known how B12 and folate might interact to influence the formation of the neural tube many fish and shellfish are among the top sources of vitamin B12 *

HYDROCEPHALUS (H)Hydrocephalus = water headAbn CSF accumulation in ventricles IC pressure in skull, progressive head enlarge-ment, convulsions & mental disabilityNormally CSF circulates through brain, ventricles, spinal cord & drains into circulatory system

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HYDROCEPHALUS (H)H caused by overproduction of CSF, congen malf, blocking, complication of trauma capitis or inflammation blockage in ventriclesAny cause of CSF accum in ventricles brain compression convulsion & retardation especially older infantsYounger infants abn large head & downward gazeTh/ shunting excess CSF to peritoneum*

HYDROCEPHALUS (H)Internal H : 4th foramina or cerebral aqueduct blocked CSF accum in ven-tricles (brain stem tu) IC press irreversible brain damageExternal H : CSF accumulation in subarach spaceH cause : CSF flow obstruction, reabsorp-tion, excessive production*

HYDROCEPHALUS (H)1.Obstruction (most common) : aqueduct stenosis, foramen Monro stenosis due to tu, inflammation, hemorrhage, congenital malformation2.Overproduction of CSF : choroid plexus papillomaH : 1.communicating 2. Non-communicating (obstructive)*

HYDROCEPHALUS (H)1.Communicating H : impaired CSF reabsorption functional impairment of arachnoidal granulations in sup sagital sinus by subarachn / IVH, meningitis, chiari malf, congen absence of Pachionis granulations Normal Pressure H (NPH), H exvacuo (enlargement of ventricles & subarach space by brain atrophy in dementia, schizophrenia)NPH : intermittent ICP D / continuous intra-ventricular pressure recording*

NORMAL PRESSURE HYDROCEPHALUS NPHNPH : Hakim triad gait instability, urinary incontinence, dementiaFocal signs : n.6 palsy, gaze palsy (Parinaud sy) due to compression of quadrigeminal plateThe increased volume of CSF press down & damage brain tissue*

RISK FACTORS OF NPHSubarachnoidal hemorrhageClosed head ijuryMeningitisSurgery of the brain (craniotomy)NPH account for 5 % of all dementiasSymptoms begin slowly *

3 SYMPTOMS OF NPH1.Walking difficulties (gait apraxia), feet held wider apart, shuffling & unsteadiness2.Slowing of mental function : forgetfulness, difficulty paying attention & apathy3.Urinary incontinence & sometimes fecal incontinence

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EXAMS AND TESTS OF NPHSudden falls without a loss of consciousness or drops attacks may occur early in the illnessWalking changes & increased deep tendon reflexes in the lower legsLumbar puncture with careful testing of walking before & after spinal tapHead CT-scan or MRI of the head

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Treatment of NPH * Ventricoperitoneal shunt* Some improve, but many do notWithout treatment, symptoms often get worse and could lead to deathPeople with minimal symptoms have the best outcomePossible complications : infection, bleeding, dementia that become worse, injury form falls, shortened life span

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HYDROCEPHALUS (H)Noncomm H / obstructive H caused by CSF obstruction :1.Foramen Monro obstruction (colloid cyst)2.Aqueduct Sylvvi ( atresia, tu, hemorrh, inflam)3.4th ventricle obstruction4. Foramen Luschka & Magendi obstr (Dandy Walker malf)5.Subarchn space surround brainstem obstruction (hemorrhage, inflammation)*

HYDROCEPHALUS (H)Congenital H : IVH in premature infants, inflam, type 2 Arnold Chairi malf, Dandy Walker malf, aqueduct atresiaSymptoms : poor feeding, vomiting, apatis, downward eyes, visual reduction, weak movements80 - 90 % infant with spina bifida associated with meningocele, myelomeningocele & develop H*

CHAIRI MALFORMATION-CMThe indented bony space at the lower rear of the skull is smaller than normal -> cerebel-lum & brainstem is pushed downward. The resulting pressure on the cerebellum can block the flow of csf & cause dizziness, muscle weakness, numbness, vision problems, headache, and problems with balance and coordination. There are three primary types of CM. *

CMMost common is Type I -> no symptoms & found by accident during examination for another condition. Type II (Arnold-Chiari malf) is accompanied by myelomeningo-cele-a form of Sp Bi -> partial or complete paralysis below the spinal opening. Type III -most serious form -> severe neurological defects. Often associated with hydrocepha-lus, syringomyelia, and spinal curvature.*

*Arnold-Chiari malformation, blocking fluid flow between the ventricles and ...www2.warwick.ac.uk

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DANDY-WALKER SYDandy-Walker Syndrome is a congenital brain malf. involving the cerebellum and the fluid-filled spaces around it -> enlargement of the fourth ventricle, a partial or complete absence of the area of the brain between the two cerebellar hemispheres (cerebellar vermis), and cyst formation near the internal base of the skull. Increase size of the fluid spaces surrounding the brain & increase in pressure -> hydrocephalus.*

*Dandy Walker is known by many names: Dandy Walker Syndrome, Dandy Walker Malformation, Dandy Walker Cyst, Dandy Walker Variant

*A sagittal T1-weighted magnetic resonance imaging (MRI) scan in a 5-year-old girl (also see the following 2 images) shows a large posterior fossa cyst elevating the torcular herophili and sinus rectus (short arrow). The hypoplastic vermis is everted over the posterior fossa cyst (long arrow). The cerebellar hemispheres and brainstem (b) are hypoplastic. Thinned occipital squama is seen (arrowheads).

*An axial T2-weighted MRI scan that shows hydrocephalus, a large cerebrospinal fluid cyst in the posterior fossa, thinned occipital bone (arrows), and hypoplastic cerebellar hemispheres with a winged appearance (c).

HYDROCEPHALUS (H)Acquired H caused by CNS inflam,brain tu, meningitis, trauma capitis, IC hemorrhSymptoms of IC press : Hache, nausea, vomit, papiledema, sleepiness, coma IC press uncal / cerebellar herniation brain stem compression life threatening*

HYDROCEPHALUS (H)H symptoms : learning disability, visual problems, clumpsy, precocious puberty, epilepsyD/ CT-scanTh / ventricular cath into ventricles drain into body cavities (peritoneum, right atrium, pleura)Shunt complication: malfunction, inflammation, failure hache, nausea, vomit, photophobia, seizures *

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