Neurology Flashcards by Diluki Rathugamage

Cerebral palsy; definition, epi, aetio.

CP is a disorder of posture and movement due to non-progressive damage to the developing brain.

Presents by the age of 3yrs, 2per 1000 live births.

Aetio
-80% are antenatal in origin (due to genetic defects)
-10% due to HIE during delivery (preterms are esp vulnerable to brain damage from periventricular leucomalacia)
-10% are postnatal (meningitis, TBI, hydrocephalus, hyperbilirubinemia…)

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Differentiating CP with other neurological conditions.

In CP, the child will not lose any milestones they’ve already gained even if motor disorder will evolve w/o Rx. (The milestones may be delayed)

In other neuro disorders, there is progressive loss of already gained milestones.

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Classification of cerebral palsy.

Spastic 90%
Dyskinetic 6%
Ataxic 4%

Hemiplegic, diplegic, quadriplegic, monoplegic & triplegic

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Categorisation of severity in CP.

Gross Motor Function Classification System (GMFCS)

I= walks w/o limitations
II= walks w/ limitations
III= walks w/ handheld mobility devices (frames)
IV= self mobility w/ limitations (may use powered ones)
V= transported in a manual wheelchair

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Spastic CP features

Damage to the UMN in the pyramidal/corticospinal tract with spasticity and exaggerated reflexes. May have initial hypotonia.

Has types; hemiplegia, diplegia, quadriplegia

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Dyskinetic CP features

Basal ganglia are affected with affection of the upper limb being more severe.

Most commonly due to HIE and kernicterus

CF are floppiness, poor trunk control and delayed motor development in infancy.

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Ataxic CP features

Parkinsonism traits in gait etc and usually affects the same side and are mostly genetically determined.

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Mx of CP

MDT consisting of parents, paediatrician, physiotherapists, occupational therapists, ENT surgeons, eye surgeons, orthopaedic surgeons, social workers, community care nurse and teachers.

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Drugs used in CP

Baclofen to reduce muscle tone
Bensexole (Artaine) to reduce dystonia
Botulinum toxin
Surgery for contractures

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Bacterial causes of meningitis in different age groups

Neonate - 3 months = GBS, E.coli, Listeria
1m- 6yrs = Neisseria meningitidis, S. pneumoniae, H. influenza
>6 yrs = Neisseria, Strept

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When to consider neuroimaging in meningitis?

When fever doesn’t settle with ABx
Signs of brain abscess
Signs of increased ICP
In focal (one-sided) seizures

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Contraindications for LP(8)

Cardiorespiratory instability
Focal neurological signs
Signs of increased ICP
Coagulopathy
Thrombocytopenia
Location infection at site of LP
Shock
No consent

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CSF findings in partially treated meningitis

Lymphocytes will be high instead of neutrophils, proteins remain high, sugar remains low and culture is -ve.

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Complications of meningitis

Hearing loss
Local vasculitis leading to cranial nerve palsies
Local cerebral infarctions
Subdural effusions
Hydrocephalus
Cerebral Abscess (vanco and drainage)

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Causes of un-resolving fever in meningities

Wrong ABx and/or dose, viral/fungal aetio, TB, formation of a brain abscess

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Common causes of encephalitis

HSV (ass. w/ temporal lobe epilepsy) Rx acyclovir IV
JE
Dengue

Clinical features of Febrile Seizures

Generalised
<15 minutes
One episode/day
No brain damage

Mx of febrile seizures

Advise parents to not out anything in the mouth during the seizure, and if it lasts more than 5 min; to hospitalise the child

Antiepileptics (eg: clobazam) is given only during fever days

Complication of Guillain-Barre Syndrome

Miller- Fischer Syndrome; ataxia, areflexia and external ophthalmoplegia

Duchenne’s muscular dystrophy; aetio, epi

X-linked recessive disease that affects the gene coding for dystrophin leading to necrosis of muscle cells and their replacement with fat

Usually present by 3-5yrs

Duchenne’s; CF

Problems in gross motor development milestones
Gower’s sign (stand using arms)
Pseudohypertrophy of calf muscles
Waddling gait
Language delay

Duchenne’s; Prognosis and complications

Poor prognosis w/o Rx; death by late 20s due to respiratory failure or ass. cardiomyopathies (dilated)
If good RX; 50-60s w/ loss of independent ambulation.
Scoliosis is another common complication

Duchenne’s; Investigations

Serum creatinine phosphokinase is markedly elevated (x1000)
Muscle biopsy
DNA testing

Duchenne’s; Mx

Medical = corticosteroids 10d each month
MDT consisting of physiotherapy, paediatricians, speech therapist, occupational therapist, orthopaedics, social worker etc

Causes of Bell's palsy in children

CP angle tumour Parotid enlargement due to mumps Forceps delivery Herpes Idiopathic

Juvenile myasthenia; patho, epi, CF

Progressive muscle weakness throughout the day due to declining levels of acetylcholine due to antibodies against ACh receptors. Presentation is usually by 10y/o w/ ophthalmoplegia and ptosis, difficulty chewing and loss of facial expression (+generalised muscle weakness).

Juvenile myasthenia; Ix, Mx

Ix = IV edraphonium over minutes followed by pyridostigmine/neostigmine over days in the HDU/ICU Mx = ACh inhibitors; pyridostigmine, neostigmine, immunosuppressive therapy, immuno-modulating drugs (MMF, prednisolone, azathioprine) Thymectomy is thymoma present Plasma exchange is used during a crisis.

Neurofibromatosis 1; genetics, criteria

De novo (50%) mutation of the neurofibromin-1 (NF-1) gene w/ autosomal dominant inheritance Criteria; >2 more of the following; ->6 cafe-au-lait spots prepuberty, >15 after puberty ->1 neurofibroma -axillary freckling -optic glioma -1 Lisch nodule (hamartoma on the iris seen on slit-lamp exam) -bony lesions from sphenoid dysplasia -1st degree relative w/ NF1

Neurofibromatosis 2; gene, CF

Less common, autosomal dominant inheritance of the NF-2 gene presenting w/ multiple inherited Schwannomas, meningiomas and ependymomas

Associated conditions w/ neurofibromatosis

MENS (endocrine disorders) Phaeochromocytoma Pulmonary HTN Renal artery stenosis

Tuberous sclerosis; cutaneous features

Depigmented ash leaf patches or a melanotic naevi (light up under UV) Shagreen patches; roughened patches of skin over the lumbar spine Angiofibromata (adenoma sebaceum) over the bridge of the nose and cheek; may be present from birth/ present at 3yrs

Tuberous sclerosis; neurological features

Infantile spasms Epilepsy (often focal) Intellectual disability (often w/ autism)

Tuberous sclerosis; other features

Subungual fibromata Phakomata (dense white areas on the retina from degeneration) Rhabdomyomata of the heart in infancy that resolves Angiomyolipomas Polycystic kidneys Cysts in lungs

Tuberous sclerosis; Mx

Seizures; vigabatrine cardiology follow-up USS KUB Special education

Sturge-Weber syndrome

Sporadic disorder w/ a haemangiomatous facial lesion (port-wine stain) in the Cr5 distribution and ass. intracranial lesion (leptomeningeal angioma) There is a high risk of ipsilateral glaucoma

DDx of meningocele vs. meningomyelocele

Meningocele; covered by skin and nerve tissue is normal Meningomyelocele; not covered by skin therefore is at a higher risk of rupture, infection and meningitis. Nerve tissue lies outside

Myelomeningocele associations

Variable paralysis of the legs Muscle imbalance Sensory loss Bladder denervation Bowel denervation Scoliosis Hydrocephalus from a Chiari malformation

Types of hydrocephalus with their relative causes.

Communicating = obstruction at the arachnoid villi (the site of absorption of CSF). - SAH, meningitis Non-communicating = obstruction w/i the ventricular system or aqueduct. - Congenital malformation (Chiari, aqueduct stenosis), posterior fossa neoplasm or vascular malformation, intraventricular haemorrhage in a preterm infant

10 features to look for in hydrocephalus

Sunsetting sign Papilloedema Cushing's reflex; HTN w/ bradycardia Examine shunt Features of shunt infection Association; meningomyelocele Hepatosplenomegaly Severity Vision, hearing, bowel and bladder function