Lecture day 2 Flashcards
(185 cards)
1
Q
What does ALS stand for?
A
Amyotrophic lateral sclerosis
2
Q
What is the median survival for someone with ALS?
A
24-50 months
10% of patients will live for 10 years
3
Q
What is the median age of onset for ALS?
A
51-66
4
Q
What is the pathophysiology of ALS?
A
TDP-43 cytoplasmic aggregates
Normally resides in nucleus. In ALS, this will move to cytoplasm and aggregate
5
Q
What is the most common genetic link in ALS?
A
C9ORF72
ALS lies on the spectrum with frontotemporal dementia
6
Q
How does ALS affect UMN and LMN?
A
Classical ALS is a combination of UMN and LMN impairment
In upper motor neuron (corticospinal tract) → impairment
- Spasticity
- Hyper-reflexia
- Babinski reflex
- Weakness
Lower motion neuron (anterior horn cell)
- Atrophy
- Fasciculations
- Hypo-reflexia
- Weakness
7
Q
What are clinical signs of ALS?
A
- Atrophy of thenar eminences and first dorsal web spaces
- Atrophy of the tongue
- Upper limb fasciculation
8
Q
What is the disease heterogeneity in onset in ALS?
A
1/3 starts as bulbar
1/3 starts lower limb
1/3 starts with upper limb
9
Q
What are typical presenting symptoms for ALS?
A
- Speech slurring or hypophonia
- Clumsiness of hands
- Foot drop
- Asymmetric at onset
- Absence of major sensory symptoms
10
Q
What are associated features of ALS?
A
- Fasciculations
- Cramps
- Gait imbalance
- Falls
- Emotional lability
- Weight loss
11
Q
What guidelines would you use to diagnose ALS?
A
El Escorial
12
Q
How long does it often take to go to onset of symptoms to diagnosis of ALS?
A
12 months
13
Q
What investigations can you do for ALS?
A
Needle electromyography: mixed pattern of acute and chronic denervation
Diagnosis remains clinical - imaging excludes mimics
14
Q
What are features of acute denervations?
A
Fibrillations, positive sharp waves and fasciculations (more precisely a sign of neuronal hyperexcitability)
15
Q
What are features of chronic denervations?
A
Large, long, polyphasic motor unit action potentials (indicating compensatory reinnervation)
16
Q
What are differential diagnoses for ALS?
A
Very few conditions cause mixed UMN and LMN damage
Syphilis is the great imitator
Sometimes vit B12 deficiency
17
Q
What diseases mimic LMN impairments?
A
Spinal degenerative disorder Multifocal motor neuropathy Myasthenia graves HIV Vitamin B12 deficiency
18
Q
What diseases mimic UMN impairments?
A
Stroke MS Neurosyphilis hereditary spastic paraplegia Leukodystrophies Vitamin B12 deficiency
19
Q
What are established treatment options?
A
Mainly riluzole
Some symptomatic treatments
20
Q
What are the benefits of Riluzole?
A
Glutamate antagonist which improves survival by 20%
21
Q
What are primary headaches?
A
Migraine
Tension-type headache
Trigeminial autonomic cephalalgias
22
Q
What are secondary headaches?
A
- Trauma or injury to the head
- Cranial or cervical vascular
- Intracranial non-vascular
- Substances
- Infection
- Homeostasis
23
Q
What are the characteristics of a migraine?
A
Typically lasts 4-72 hours
Unilateral, pulsating, moderate to severe, aggravated by daily activity
Nausea/vomiting or photophobia and phonophobia
24
Q
What are the characteristics of a cluster headache?
A
5-180 minutes
Severe or very severe unilateral orbital or temporal
Often with infection, eyelid oedema, nasal congestion, forehead fo facial sweating, mitosis or ptosis
Between 8 per day to one every other day
25
What are red flags for headaches?
1. Systemic symptoms (fever or weight loss)
2. Altered consciousness
3. Age over 50 (unusual to have sudden onset headaches - more likely to be secondary)
4. Postural or positional (CSF venus fistulla)
5. Precipitated: Valsalva, exertion, sex
6. Papilloedema
26
What are the differentials for a thunderclap headache?
1. Subarachnoid haemorrhage
2. Intracerebral haemorrhage
3. Cerebral venous thrombosis
4. Arterial dissection
5. Reversible cerebral vasoconstriction syndrome
6. Pituitary apoplexy
27
What are the characteristics of a raised ICP headache?
1. Worse in morning
2. Better in upright
3. Worse with Valsalva
28
What are the characteristics of a lower ICP headache?
Worse as day goes on
| Better recumbent
29
What are the characteristics of idiopathic intracranial hypertension?
Persistent, dull, pressing bilateral headache. Aggravated by coughing, pressing and physical activity. Potential nausea
Visual disturbances: decrease in acuity, increase in blind spot, papilloedema
30
How would you treat migraine?
1. NSAIDs
2. Triptans (use when patient is not pain free after 2 hours on NSAIDs)
3. Antiemetics (occasionally, but not often)
1. Metoclopramide
2. Domperidone
Preventive treatment (use when around 7 migraines per month)
31
What preventative treatment could you give for migraines?
Metoprolole
Valproate
Flunarizine
32
What is a medication overuse headache?
- Headache occurring on ≥ 15 days/month in a patient with a pre-existing headache disorder
- Regular overuse for > 3 months of one or more drugs that can be taken for the acute and/or symptomatic treatment of headache
33
How would you manage a cluster headache?
1. 100% oxygen (12-15 l/min) (after 10 minutes the headache is gone)
2. Triptans (sc or nasal)
34
When would you give preventative treatment in a cluster headache?
ALWAYS
35
What are the preventative treatments for cluster headaches?
1. Verapamil (works most of the time)
| 2. Topiramate, lithium
36
What are possible causes of tingling limbs?
1. Stroke or TIA
2. Spinal cord pathology
3. Spinal nerve compression
4. Peripheral nerve compression
6. Nerve damage due to trauma
7. MS
8. Endocrine
9. Vitamin deficiency
10. Nerve toxicity
37
What can cause spinal cord compression?
metastatic malignancy, herniated disc
38
What can cause peripheral cord compression?
Tumour, enlarged blood vessels, infection
39
What endocrine disturbances can cause tingling limbs?
Diabetes
| Hypothyroidism
40
What vitamin deficiencies can cause tingling limbs?
Vitamin B12
| Thiamine
41
What substances can cause nerve toxicity?
Alcohol
| Lead
42
What is hyperpathia?
Heightened noxious sensation
43
What is allodynia?
Noxious response to non-noxious stimuli
44
What is the MRC scale?
Muscle Power Scale
45
What are the grades of the MRC scale?
Grade 0 - no muscle movement
1. Grade 1 = flicker or trace of muscle movement
2. Grade 2 - active movement with gravity
3. Grade 3- active movement against gravity
4. Grade 4 = active movement against gravity and resistance
5. Grade 5 = normal power
46
What kind of lesions would result in muscle atrophy?
UMN
47
What kind of lesions would result in muscle wasting?
LMN
48
What kind of lesions would result in Babiski sign?
UMN
49
What kind of lesions would result in hyperreflexia?
UMN
50
What kind of lesions would result in hyporeflexia?
LMN
51
What kind of lesions would result in a foot drop?
LMN
52
What kind of lesions would result in fasciculations?
LMN
53
What muscle, nerve and root are responsible for shoulder abduction?
Deltoid
Axillary
C5
54
What muscle, nerve and root are responsible for elbow flexion?
Biceps
Musculocutaneous
C5, C6
Brachioradialus
Radial
C6
55
What muscle, nerve and root are responsible for elbow extension?
Triceps
Radial
C7
56
What muscle, nerve and root are responsible for radial wrist extension?
Extensor carpi radialis longus
Radial
C6
57
What muscle, nerve and root are responsible for finger extension?
Extensor digitorum communis
Posterior interosseeous nerve
C7
58
What muscle, nerve and root are responsible for finger flexion?
Flexor pollicis longus
Anterior interosseous
C8
Median nerve C8
Flexor digitorum profundus
Ulnar (ring and little finger)
59
What muscle, nerve and root are responsible for finger abduction?
First dorsal interosseous and abductur digiti minimi
Ulnar
T1
Abductor pollicis brevis
Median
T1
60
What muscle, nerve and root are responsible for hip flexion?
Iliopsoas
| L1 L2
61
What muscle, nerve and root are responsible for hip adduction?
Adductors of the hip
Obturator
L2, L3
62
What muscle, nerve and root are responsible for hip extension?
Gluteus maximus
Sciatic
L5, S3
63
What muscle, nerve and root are responsible for knee flexion?
Hamstring
Sciatic
S1
64
What muscle, nerve and root are responsible for knee extension?
Quadriceps
Femoral
L3, L4
65
What muscle, nerve and root are responsible for ankle dorsiflexion?
Tibialis anterior
Deep peroneal
L4
66
What muscle, nerve and root are responsible for ankle eversion?
Peronei
Superficial peroneal
L5, S1
67
What muscle, nerve and root are responsible for ankle plantar flexion?
Gastrocnemius, soleus
Tibial
S1, S2
68
What muscle, nerve and root are responsible for big toe extension?
Extensor hallucis longus
Deep peroneal
L5
69
What investigations would you do for tingling limbs?
1. Blood tests
2. HIV test
3. Environmental exposure
4. Nerve conduction studies
5. EMG
70
What is the function and velocity of type alpha fibres?
Proprioception, somatic motor
70-120 m/s
71
What is the function and velocity of type beta fibres?
Touch and pressure
30-70 m/s
72
What is the function and velocity of type gamma fibres?
Motor to spindle muscles
15-30 m/s
73
What is the function and velocity of type delta fibres?
Pain, cold, touch
12-30 m/s
74
What is the function and velocity of type B fibres?
Preganglionic automatic
3-15 m/s
75
What is the function and velocity of type C dorsal root fibres?
Pain, temperature, mechanoreception
0.5-2 m/s
76
What is the function and velocity of type C sympathetic fibres?
Postganglionic sympathetic
0.7-2.3 m/s
77
What are key features of length dependent neuropathies?
1. Symptoms start distally (often fingers)
2. Often diabetes
3. Usually symmetrical
4. Motor signs: loss of reflexes and wasting
5. Sensor signs: reduced pain, temp, vibration and proprioception
78
What investigations would you do for length dependent neuropathies?
Full blood
ANA, HIV, ANCA, anti-neuronal antibodies
EMG
Microneuropathy
Nerve biopsy
79
What are key features of entrapment neuropathies?
1. Common world-wide and occupation related
2. Mechanical or dynamic compression of the nerve
3. Symptoms progress slowly and include pain, sensory loss, tingling and motor loss
4. Treatment includes splinting, steroids, surgery
80
What is neuropraxia?
Loss of myeline
81
What are key facts about carpal tunnel?
Often due to pregnancy, myxoedema, wrist injury or genetics
Symptoms in the expected distribution of median nerve
Relieved by movement, worse in the morning
82
How would you treat carpal tunnel syndrome?
1. Splinting
2. Steroid
3. Surgery
83
What are the main differentials besides carpal tunnel?
C8 radiculopathy
| C8 plexopathy
84
If the ulnar intrinsic muscles are involved, where is the problem?
Ulnar nerve: wrist or elbow
85
If the ulnar intrinsic AND ulnar forearm muscles are involved, where is the problem?
Elbow
86
If the ulnar intrinsic AND ulnar forearm AND median intrinsic muscles are involved, where is the problem?
C8 or T1 radiculopathy or brachial plexus lesion
87
If there is a sensory abnormality in the terminal digits only, where is the problem?
Wrist or elbow
88
If there is a sensory abnormality in the terminal digits AND palmar cutenaeous/dorsal ulnar cutaneous, where is the problem?
Elbow
89
If there is a sensory abnormality in the terminal digits AND palmar cutenaeous/dorsal ulnar cutaneous AND inner forearm, where is the problem?
C8 or T1 radiculopathy or brachial plexus lesion
90
What are causes of ulnar neuropathy?
1. Trauma
2. Elbow bony deformity
3. External pressure (leaning on elbow)
91
What muscles are involved in foot drop?
Dorsiflexors
Common peroneal nerve palsy at the fibular head
92
How would you manage foot drop?
Conservatively
93
What are differentials for foot drop?
Common peroneal nerve palsy at the fibular head
L5 radiculopathy post partum
UMN lesions
94
What are the clinical findings of common peroneal palsy?
Weakness of foot dorsiflexion, eversion, and toe extension
Sensory loss of lateral lower leg and dorsum of the foot
95
What are the clinical findings of lumbar plexopathy?
Weakness of hamstrings, glutei, foot inversion
Sensor loss is variable and difficult to delineate
96
What are the clinical findings of lumbrosacral trunk of the plexus?
Weakness of hamstrings, glutei, foot inversion
Patch sensory loss
97
What are the clinical findings of sciatic neuropathy (L4-S1)?
Mimics peroneal palsy
Sensory loss of the dorsum of the foot and lateral side of lower leg
98
What are the clinical findings of peripheral neuropathy?
Stronger dorsiflexion than plantar flexion (heel toe test)
Length dependent pattern of sensory loss
99
What are the clinical findings of L4/L5 radiculopathy?
Pain in L5 dermatome
Weakness of EHL, toes extensor, foot eversion, hip abduction
100
What are the clinical findings of L5/S1 radiculopathy?
VERY common
Pain in back of leg and sole of foor
Ankle jerk reduced, weakness of plantar flexion may be present
101
What can be a precipitating factor for radiculopathy?
Acute pain
102
What are findings for radiculopathy?
Weakness of muscles innervated by the root
Absent or reduced reflexes
Dermatomal sensory loss
103
What investigations would you do for radiculopathy?
Usually none
For atypical one: MRI
104
How would you treat radiculopathy?
Conservatively
Physio, bed rest, analgesia
Takes 6 weeks to settle
105
When would you consider surgery for radiculopathy?
Unremitting pain
Progressive signs
Fix with root decrompression
106
What are symptoms and signs of Cauda Equina?
1. Leg weakness and perineal numbness
2. Loss of bladder and erectile functions
3. Change in bowel function
4. Lower motor flaccidity, weakness and areflexia
107
What are causes of extrinsic cord lesions?
Tumour, compression
108
How do signs and symptoms progress in extrinsic cord lesions?
Motor symptoms and signs tend to predominate and come early
Sensory signs may evolve later and develop rapidly
Bladder and bowel often involved LATE
109
How do signs and symptoms progress in intrinsic cord lesions?
Bladder and bowel often involved EARLY
Causes spontaneous pain
110
What are inherited neuropathies?
CMT: demyelinating (CMT1) or axonal (CMT2)
111
What are key facts of CMT1?
Duplication of PMP22 gene on chromosome 17
Length depending neuropathy: wasting of peronei
Distal weakness
112
What is meralgia paraesthetica?
Entrapments of the lateral cutaneous nerve of the thigh, often in obesity, diabetes and ageing population
113
How does meralgia paraesthetica present?
Pain, allodynia and numbness
Sensory loss lateral to midline of the thigh, no motor findings
Clinical diagnosis
114
How would you manage meralgia paraesthetica?
Weight loss
Remits over time
115
What are differential diagnosis for wrist drop?
Posterior interosseous nerve at the arcade of Frohse
Causes finger weakness and no sensory signs!!
116
What is Churg Strauss syndrome?
The disorder is characterised by the abnormal hypereosinophilia in the blood and tissues and vasculitis
Presents with asthma, hayfever, rash and sinusitis
117
How does Churg Strauss syndrome present?
Eosinophilia: fevers, weight loss, arthralgia, breathlessness, neurological signs
Patients often left with significant neurological impairments
118
How would you manage Churg Strauss syndrome?
Treatment of vasculitis and intensive suppression of the immune system
119
How does sudden sensorineural hearing loss present?
1. Tinnitus
2. Sudden onset
3. Feeling of fullness in ears
4. Unilateral
5. Sensorineural based on tuning fork test
120
How would you manage sensorineural hearing loss?
1mg/kg prednisolone per day
- Start within three days of onset
- Refer to ENT emergency clinic
121
What is the most dangerous foreign body?
Battery
122
How would you remove an insect from the ear?
OIL ! Kills it
123
What is the main problem with foreign bodies in the ear?
Main danger is clumsy attempt at removing it
A large foreign body may cause temporary hearing loss
124
How does otitis externa present?
1. Pain (severe)
2. Discharge
Often in swimmers or after local trauma?
125
How would you manage otitis externa?
- Topical antibiotic/steroid drops
- Keep water out!
- Refer if doesn’t resolve
126
What is acute otitis media often caused by?
- H influenzae
| - S pneumoniae
127
How does acute otitis media often present?
Acute inflammation of the middle ear
1. Pain
2. Discharge
In children
128
What are the complications of acute otitis media?
1. Meningitis
2. Abscesses
3. Venous thrombosis
If left untreated it can cause perforation
129
What are causes of facial nerve palsy?
- Congenital
- Inflammation
- Acute otitis media
- Bell’s palsy
130
What are classic symptoms of Bell's palsy?
- Inability to close eye
- Downwards angle of mouth
- Disordered movement of the muscles that control facial expressions, such as smiling, squinting, blinking, or closing the eyelid
- Drooling
- Loss of feeling in the face.
- Loss of the sense of taste on the front two-thirds of the tongue.
- Asymmetrical smile
- No muscle tone in cheek
- Cannot furrow brow
131
How would you treat Bell's palsy?
1. Steroids (oral corticosteroids)
| 2. Eyecare
132
If you think something is Bell's palsy, but it's not. What can it be?
1. Granulomatous disease – WG, Sarcoid, TB
2. Parotid malignancy – adenoid cystic Ca
3. Malignant OE (skull base osteomyelitis)
4. Ramsey Hunt Syndrome
5. Lyme disease
6. HIV
7. Facial neuroma
8. Vestibular Schwannoma
133
What are signs of skull base fracture?
- Bruising behind the ears
- Bruising around the eyes
- Blood behind the ear drum
Types include anterior, central and posterior. Due to severe head injury
134
What are complications of skull base fracture?
- Cerebrospinal fluid leak
- Facial fracture
- Meningitis
135
What causes temporal bone fracture?
Traumatic head injury
Penetrating injury
Falls
136
What are signs of temporal bone fractures?
Anterior: raccoon eyes, halo signs, partial loss of vision and smell, eye movement defects
Middle: damage to carotid, hearing loss, loss of balance, battler sign
Posterior: cervical spine injury, vertebral injury, damage to lower cranial nerves
137
How would you diagnose temporal bone fractures?
Physical exam
Glasgow coma scale
Neurological examination
CT
138
What is the nasal blood supply?
1. External carotid
1. Sphenopalatine artery
2. Greater palatine artery
3. Ascending pharyngeal artery
4. Posterior nasal artery
5. Superior labial artery
2. Internal carotid
1. Anterior ethmoid artery
2. Posterior ethmoid artery
139
What do you know about anterior versus posterior nose bleed?
1. Anterior
- Most common (over 90%)
- Usually in younger patients
- Usually septal
- Typically less severe
2. Posterior
- Older population
- Usually Woodruff’s plexus
- Typically more serious
140
What are causes of nosebleed?
1. Local: trauma, foreign bodies, neoplasms, idiopathic
| 2. Systemic: medications, ethanol, HHT, coagulapathy
141
What are non-surgical ways to treat nosebleeds?
1. Control hypertension
2. Correct coagulopathies/thrombocytopenia
3. Expel clots
4. Topical decongestants or vasoconstrictors
5. Cautery
6. Nasal packing
142
What are surgical ways to treat nosebleeds?
1. Endoscopic sphenopalatine artery ligation
2. Transmaxillary maxillary artery ligation
3. Anterior/posterior ethmoid artery ligation
4. External carotid artery ligation
5. Embolization
143
What are signs of Acute rhino-sinusitis?
- Pain exacerbated by bending forwards
- No cheek swelling
- Fever
- Nasal purulence
- Raised CRP
144
How would you manage Acute rhino-sinusitis?
- Analgesia
- Steam inhalation
- Decongestants
- ephidrine
- Otrivine
- Antibiotics
145
How would you treat a complicated Acute rhino-sinusitis?
1. Urgent CT with contrast
2. Orbital collections and urgent drainage
3. Intracranial collection, urgent neurosurgical drainage
4. Antibiotics, decongestants, washout sinuses
146
What are complications of sinusitis?
1. Orbital cellulitis
2. Frontal osteomyelitis
3. Meningtitis
4. Cerebral abscess
5. Cavernous sinus thrombosis
147
Where would you do a cricothyroidotomy?
Through cricothyroid membrane between cricoid and thyroid cartilage
148
What are indications for cricothyroidotomy?
- Inability to intubate
- Inability to ventilate
- Inability to maintain SpO2 >90%
149
How would you lower intracranial pressure?
1. Elevate head of bed to 30 degrees
2. Discontinue cervical collar
3. Straightedges head and neck to neutral position
4. Remove ties around neck
5. Treat fever
150
What are the brainstem reflexes that are tested to confirm brainstem death?
1. Pupil response
2. Corneal reflex
3. Oculovestibular reflex
4. Apneic test
151
How would you confirm brainstem death?
1. Comatose patient dependent on mechanical ventilation
2. Irreversible brain damage of known aetiology
3. Exclusion of reversible causes of coma and apnea: drugs, hypothermia, CVD/metabolic/endocrine disturbances
4. Absent brain stem reflexes, absent motor reflexes in cranial nerves
5. Confirm death
152
What does space occupation in the CNS lead to?
e.g. tumour, blood, swelling
1. Focal deficits
2. Seizures
3. Raised ICP
153
Who can you use brain US for?
Used in neonatal setting for many years for brain and spine due to open fontanelles
Increasing use intraoperatively
154
What are the different types of hydrocephalus?
- Communicating (post-haemorrhagic; post-infection)
- Obstructive (aqueduct stenosis; obstruction at the outlets of the
4th ventricle; foramina of Monro; tumour; blood; infection; membranes and cystic lesions)
155
How does hydrocephalus present in children?
1. Increasing head circumference
2. Full and tense fontanelle
3. Dilated scalp veins
4. Loss of upgaze
5. Irritability
6. Vomiting
7. Drowsiness, reduced conscious level
156
How does hydrocephalus present in adults?
1. Headaches
2. Nausea and vomiting
3. Visual blurring
4. Drowsiness, reduced conscious level
157
How would you manage hydrocephalus?
1. CSF diversion
2. Temporary → reservoir or VSGS
3. Definitive → shunt, ETV
158
How would you classify trauma based on GCS?
- Mild (13-15)
- moderate (9-12)
- severe (3-8)
159
What are contusions?
Traumatic intracerebral haematomas
Multiple small contusions (petechial haemorrhages) are one of the signs of diffuse axonal injury
160
What are risk factors for a subarachnoid haematoma?
Smoking, HTN, family history, connective tissue disorder
161
How would you manage a subarachnoid haematoma?
Endovascular coiling and neurosurgical clipping
Urgent !
162
What is a cavernoma?
Cluster of abnormal blood vessels, usually found in the brain and spinal cord
163
How do cavernoma often present?
Seizures, bleeds
164
What are causes of spine injury?
1. Degenerative (UMN, radiculopathy, spine degeneration)
2. Tumour
3. Trauma
165
How would you grade spinal lesions?
Grade A: complete: no sensory or motor function
Grade B: incomplete. Sensory but no motor function
Grade C: incomplete. Motor function is preserved below the neurological level
Grade D: incomplete. Similar to C but more preserved muscles
Grade E: normal
166
What is pituitary apoplexy?
Expansion of pituitary adenoma due to haemorrhage or ischaemic necrosis
167
How does pituitary apoplexy present?
Sudden and rapid onset of severe headache, nausea, vomiting and meningism
Visual loss (bilateral hemianopia and/or VA reduced
Hypothalamic dysfunction
168
How would you manage pituitary apoplexy?
Cortisol
169
How would you manage a prolactinoma (pituitary tumour)?
Dopamine agonists: cabergoline, bromocriptine
170
How would you manage acromegaly (pituitary tumour)?
```
Dopamine agonists
Somatostatin analogues (octreotide)
GH antagonists (pegvisomant)
```
171
How would you manage a Cushing's?
Adrenal steroid blockers (ketoconazole, metyrapone)
172
How does prolactinoma present?
Galactorrhoea, amenorrhoea in women
Impotence, loss of libido and fertility in men
173
How does a GH secreting tumour present?
Gigantism in prepubertal children
Acromegaly in adults
Skeletal overgrowth and soft tissue swelling
Glucose intolerance, peripheral nerve entrapment syndrome, headaches, excessive perspiration, sleep apnoea, fatigue
174
What are key facts about acoustic neuroma?
Unilateral sensorineural hearing loss
Bilateral tumours in type 2 neurofibromatosis
Surgery
175
When would you consider surgery for epilepsy?
After two trials for anticonvulsants
176
What diseases can you use deep brain stimulation for?
1. Parkinson's
2. Dystonia
3. Tremor
177
What are chair malformations?
a condition in which brain tissue extends into the spinal canal
178
What is the difference between type 1 and 2 chiari malformations?
Type 1 Chiari malformation symptoms and signs can show up in infants, children, teens or adults.
Type 2 Chiari malformation is associated with spina bifida and is present at birth
179
What are symptoms of type 1 chiari malformations?
Suboccipital headaches
Bilateral hearing loss
Dizziness, vertigo, disturbance of hearing
Problems of the spinal cord present in about 95% of patients with syringomyelia and two thirds of those without it. Symptoms include central cord syndrome, impaired sensation, impaired motor control, gait disturbance, torticollis, bowel and/or bladder symptoms, neuropathic joints, trophic phenomena and pain
180
What are symptoms of type 2 chiari malformations?
```
Inspiratory stridor.
Dysphagia or nasal regurgitation.
Aspiration from bilateral abductor vocal cord paralysis or central neural dysfunction, or both.
Respiratory distress.
Episodes of apnoea.
Weak cry.
Scoliosis.
Quadriparesis.
```
181
What investigations would you do for chair malformations?
MRI
182
What is the management for Chiari?
Surgery
183
What are the different types of neural tube defects?
1. Open: spina bifida and myelomeningocoele
| 2. Closed: fatty film, dermal sinus tract, lipoma, neurenteric cyst
184
How would you manage open neural tube defects?
Repaired surgically
185
How would you manage closed neural tube defects?
Only intervene when symptomatic
