Bilateral spastic paralysis (spastic paraplegia)

Published on 02/04/2015 by admin

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36 Bilateral spastic paralysis (spastic paraplegia)

Instruction

Carry out a neurological examination of this patient’s lower limbs.

Salient features

Examination

• Increased tone in both lower limbs

• Hyperreflexia

• Ankle clonus

• Weakness in both lower limbs

• Then proceed to:

• Check the sensory level

• Examine the spine (spinal tenderness or deformity).

• Tell the examiner that you would like to do the following:

• Check sacral sensation

• Examine the hands to rule out involvement of upper limbs

• Check for cerebellar signs (multiple sclerosis, Friedreich’s ataxia).

• Try to localize the level of lesion using the following:

• Spasticity of the lower limb alone: lesion of thoracic cord (T2–L1)

• Irregular spasticity of lower limbs with flaccid weakness of scattered muscles of lower limbs: lesion of lumbosacral enlargement (L2–S2)

• Radicular pain: useful early in the disease, with time becomes diffuse and ceases to have localizing value

• Superficial sensation: not good for localizing as the level of sensory loss may vary greatly in different individuals and in different types of lesions.

Diagnosis

This patient has bilateral spastic paraparesis (lesion) at L1 spinal level caused by trauma (aetiology); it is complicated by bladder involvement (functional status).

Advanced-level questions

What intracranial cause for spastic paraparesis do you know?

Parasagittal falx meningioma.

What do you know about transverse myelitic syndrome?

• Causes include trauma, compression by bony changes or tumour, vascular disease.

• All the tracts of the spinal cord are involved.

• The chief clinical manifestation is spastic or flaccid paralysis.

• The lesion can be incomplete cord compression or total cord transection.

	Total cord transection	Incomplete cord compression
Paraplegia in flexion	+	+
Paralysis	Symmetrical	Asymmetrical
Flexor–withdrawal reflex	+ without return (withdrawal phase only)	Associated with return to original position
Other	Vasomotor and sphincter changes	Variable area of anaesthesia that is not consistent with motor loss

What do you know about paraplegia-in-flexion?

Paraplegia-in-flexion is seen in partial transection of the cord where the limbs are involuntarily flexed at the hips and knees because the extensors are more paralysed than the flexors. In complete transection of the spinal cord, the extrapyramidal tracts are also affected and hence no voluntary movement of the limb is possible, resulting in paraplegia-in-extension.

Where is the lesion in patients with spastic weakness of one leg?

The lesion may be localized to the spinal cord or the brain. Progression to involve the arm does not help to differentiate between the spinal cord or the brain. Similarly, spread to the opposite leg does not necessarily indicate that the lesion is the spinal cord. Full investigation would include radiography of the spine, CT and MRI with spinal angiography to exclude spinal dural arteriovenous fistula.

What do you know about hereditary spastic paraplegia?

This is an autosomal dominant condition, first described by Seeligmuller and Strumpell, in which spasticity is more striking than muscular weakness. The age of onset is variable and the condition has a relatively benign course. When the onset is in childhood, there may be shortening of the Achilles tendon, often requiring surgical lengthening. There is usually no sensory disturbance. An autosomal dominant form of hereditary spastic paraplegia is associated with mutations in the mitochondrial import chaperonin HSP60 (Am J Hum Genet 2002;70:1328–32). An autosomal recessive form of hereditary spastic paraplegia is the result of mutations in the gene SPG7, which encodes paraplegin, a mitochondrial protein similar to yeast metalloproteases (Cell 1998;93:973–83).

What do you know about tropical spastic paraplegia?

This is seen in Japan, the Caribbean and parts of western Africa and South America where women, more often than men, in their third and fourth decades have spastic paraparesis with neurogenic bladder. Viral infection with human T-lymphotrophic virus 1 has been implicated as a cause of this disorder.

How do you localize the lesion to the second and third lumbar root level?

• Muscular weakness: hip flexors and quadriceps

• Deep tendon reflexes affected: knee jerk

• Radicular pain/paraesthesia: anterior aspect of thigh, groin and testicle

• Superficial sensory deficit: anterior thigh.

How do you localize the lesion to the fourth lumbar root level?

• Muscular weakness: quadriceps, tibialis anterior and posterior

• Deep tendon reflexes affected: knee jerk

• Radicular pain/paraesthesia: anteromedial aspect of the leg

• Superficial sensory deficit: anteromedial aspect of the leg.

How do you localize the lesion to the fifth lumbar root level?

• Muscular weakness: hamstrings, peroneus longus, extensors of all the toes

• Deep tendon reflexes affected: none

• Radicular pain/paraesthesia: buttock, posterolateral thigh, anterolateral leg, dorsum of foot

• Superficial sensory deficit: dorsum of the foot and anterolateral aspect of the leg.

How do you localize the lesion to the first sacral root level?

• Muscular weakness: plantar flexors, extensor digitorum brevis, peroneus longus and hamstrings

• Deep tendon reflexes affected: ankle jerk

• Radicular pain/paraesthesia: buttock, back of thigh, calf and lateral border of the foot

• Superficial sensory deficit: lateral border of the foot.

How do you localize the lesion to the lower sacral root level?

• Muscular weakness: none

• Deep tendon reflexes affected: none (but anal reflex impaired)

• Radicular pain/paraesthesia: buttock and back of thigh

• Superficial sensory deficit: saddle and perianal areas.

What is the characteristic type of diplegia in cerebral palsy?

Diplegia associated with prematurity is a striking clinical entity—striking for the symmetry of neurological signs, for their distribution, for the relatively good intelligence of the patients and for the comparative absence of seizures; the disability is often purely motor without sensory deficits.

What surgical treatment is available for the management of spastic diplegia in cerebral palsy?

Dorsal rhizotomy may be beneficial in selected patients.

What are the clinical features of spinal cord compression from epidural metastasis?

The initial symptom is progressive axial pain, referred or radicular, which may last for days to months. Recumbency frequently aggravates the pain, unlike the pain of degenerative joint disease where it is relieved. Weakness, sensory loss and incontinence typically develop after the pain. Once a neurological deficit appears, it can evolve rapidly to paraplegia over a period of hours to days. In suspected cases, MRI of the spine must be done by the next day. About 50% of cases in adults arise from breast, lung or prostate cancer. Compression usually occurs in the setting of disseminated disease. It is at the thoracic level in 70% of cases, lumbar in 20% and cervical in 10%, and it ccurs at multiple, non-contiguous levels in less than half of the cases. The tumour usually occupies the anterior or anterolateral spinal canal. CSF findings are non-specific in metastatic epidural compression. The cell count is usually normal, but protein levels may be raised because the flow of CSF is impeded. Lumbar puncture has been known to worsen the neurological deficit, presumably caused by impaction of the cord.

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