Spastic Paraparesis Flashcards
Spastic Paraparesis
Spastic paraparesis describes a upper motor neuron pattern of weakness in the lower limbs
Spastic Paraparesis - Causes
Causes
- demyelination e.g. multiple sclerosis
- cord compression: trauma, tumour
- parasagittal meningioma
- tropical spastic paraparesis
- transverse myelitis e.g. HIV
- syringomyelia
- hereditary spastic paraplegia
- osteoarthritis of the cervical spine
Tropical Spastic Paraparesis - Diagnosis: Example Question
A 32 year old presents with a 6 month history of progressive lower limb weakness and suprapubic tenderness. On examination, cranial nerves and upper limbs were unremarkable. You note 1/5 power in both lower limbs, hyper-reflexic patella and ankle reflexes, bilateral clonus and upgoing plantar reflexes. Abdominal examination also revealed a suprapubic mass. After a urethral catheter was inserted, you note a residual volume of 1.8l. She is known to be HIV positive, diagnosed 8 years ago. Blood tests from an outpatient appointment revealed:
CMV IgG positive EBV IgG positive HTLV antibody positive Hepatitis B negative Hepatitis C negative
An MRI spine is awaited. What is the likely diagnosis?
> Tropical spastic paraparesis Spinal toxoplasmosis CMV transverse myelitis EBV transverse myelitis Tabes dorsalis
This is a HIV and HTLV-1 positive patient presenting with paraparesis and urinary retention, a classic presentation of tropical spastic paraparesis. Toxoplasmosis in HIV patients typically present in the brain, lungs or as a chorioretinitis. Transient viral inflammatory transverse myelitis by CMV and EBV typically resolves within 3 months, the presence of IgG in both suggest previous exposure and is of limited significance. Tabes dorsalis is a possibility but peripheral reflexes are typically lost with an extensor plantar reflex. Diagnosis of HTLV-1 tropical spastic pararesis relies on a combination of clinical features and HTLV-1 load in serum and CSF. Treatments are limited, with steroids, presumably as an anti-inflammatory, producing only mild effects in a small population of patients.
Tropical Spastic Paraparesis - Example Question
A 29-year-old man has developed a gradual onset of bilateral leg weakness over the last 24 months such that he now needs to walk with crutches. He is also intermittently incontinent of urine. He will also often fall over in the dark because he feels his balance is worse then. He has never noticed any other symptoms prior to these. He has no significant past medical conditions. He works as an accountant. He went travelling for some years in his early 20’s to the Carribean, Japan and Africa. He admits occasional intravenous drug use whilst travelling and getting a tattoo. He also has casual sexual contact with sex workers occasionally whilst travelling and does not recall using barrier contraception. On examination, you find hyperreflexia bilaterally in the legs with upgoing plantar responses. He has loss of vibration and joint position sense in the legs. Legs are 3/5 power in the knee and plantar extenders and 4/5 in the flexors. He has a stomping gait. Routine blood tests plus HIV screening come back unremarkable. MRI brain and whole spine show areas of demyelination in the lumbar spine. Which test would you do to confirm the most likely suspected cause of his symptoms?
Serm JC virus levels > Serum and CSF HTLV-1 antibody levels Syphilis serology Hepatitis B serology Paired serum and cerebrospinal fluid (CSF) oligoclonal band levels
The diagnosis is tropical spastic paraparesis (TSP).
This is otherwise called HTLV-1 Associated Myelopathy (HAM), describing how this demyelinating condition is caused by infection with HTLV-1. HTLV-I is a retrovirus endemic in southern Japan, equatorial Africa and South America. Transmission occurs through sexual or other intimate contacts, intrauterine, breastfeeding, sharing of needles by drug users, or blood transfusion from infected persons. This patient has many of these risk factors from his time travelling.
The incubation period between infection and symptomatic disease may be a few months up to decades and actually, the majority of infected individuals remain lifelong asymptomatic carriers. It is not clear what determines whether or not one becomes symptomatic but the key is suspected to be bigger viral loads resulting in progression to symptoms.
The presentation is with progressive upper motor neurone symptoms and signs particularly confined to the lower limbs (see below for differentials of such a presentation.
The key to knowing which of these differentials is the likely cause here is understanding the risk factors and ordering the correct tests (e.g. not missing out an HIV test); as the signs and symptoms are fairly similar in all of these upper motor neurone conditions.
Tropical Spastic Paraparesis
Spastic paraparesis (progressive UMN signs) in the lower limbs + urinary retention in an HTLV-1 positive (and poss HIV +ve) patient > a classic presentation of tropical spastic paraparesis.
Diagnosis of HTLV-1 tropical spastic pararesis relies on a combination of clinical features and HTLV-1 load in serum and CSF. Treatments are limited, with steroids, presumably as an anti-inflammatory, producing only mild effects in a small population of patients.
This is otherwise called HTLV-1 Associated Myelopathy (HAM), describing how this demyelinating condition is caused by infection with HTLV-1. HTLV-I is a retrovirus endemic in southern Japan, equatorial Africa and South America. Transmission occurs through sexual or other intimate contacts, intrauterine, breastfeeding, sharing of needles by drug users, or blood transfusion from infected persons.
The incubation period between infection and symptomatic disease may be a few months up to decades and actually, the majority of infected individuals remain lifelong asymptomatic carriers. It is not clear what determines whether or not one becomes symptomatic but the key is suspected to be bigger viral loads resulting in progression to symptoms.
