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Progress
A few days later , Jack re-presents feeling worse . He now has hand weakness and clumsiness , his legs are numb and he has pins and needles in all extremities .
His vital signs remain normal . Neurologically , the left facial paresis has improved , but there is now weakness in both hands and wrists , reduced sensation in a bilateral stocking distribution , and absent knee and ankle reflexes .
Jack is referred back to ED with a provisional diagnosis of Guillain-Barre syndrome ( GBS ). Repeat pathology shows he now has a mild neutrophilia . The ED again discharges Jack to the care of his GP to refer to neurology outpatient care .
Unsatisfied with the management plan , the GP liaises with a local neurologist who arranges admission via a private ED . A lumbar puncture shows elevated CSF protein , in an acellular tap with a negative culture .
The neurologist agrees that GBS is the likely diagnosis and commences IV immunoglobulin . The papular rash is thought to be secondary to prednisone use .
Nerve conduction studies of the lower limbs are normal and upper limbs demonstrate slowing at the median and ulnar nerves that is reported to be potentially consistent with GBS .
Discussion
GBS is an acute or sub-acute demyelinating polyradiculoneuropathy , typically causing an acute motor axonal neuropathy , although in some cases resulting in motor-sensory deficits . The exact cause is unknown ; however , 50-70 % of cases appear 1-2 weeks after an infective illness , typically respiratory or gastrointestinal . 1 Campylobacter jejuni is the most common infective precipitant , and other associated infections that have been identified include CMV , influenza , HIV , HSV and COVID- 19 . 2 Other triggers include routine vaccination ,
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surgery , trauma and medications such as isotretinoin , tacrolimus and immune checkpoint inhibitors . 2
Symptoms develop within days to weeks , usually peaking within 2-4 weeks . 2 The typical presentation is with rapidly progressive bilateral weakness . Usually this is symmetrical and begins in the lower limbs , with about 10 % of cases manifesting first in the upper limbs or facial muscles . 2 Facial nerve palsies occur in about 50 % of cases . 2 Around 90 % of patients have decreased or absent deep tendon reflexes at presentation . 2 More than 80 % report paraesthesias in the extremities , often with normal objective sensory examination findings . 2 Pain may be a feature , usually in the back and extremities , thought to result from nerve root inflammation , and dysautonomia may also occur . 2
The initial diagnosis is clinical , supported by diagnostic testing including CSF analysis , brain imaging and electrodiagnostic studies , which also play a key role in excluding differential diagnoses . CSF typically reveals elevated protein with a normal white blood cell count . 2
Management is supportive until the recovery trajectory is established , typically after four weeks . Critical care , cardiac monitoring and thromboprophylaxis may be required in cases of significant immobility , dysautonomia or respiratory paralysis .
Immunotherapy , in the form of IV immunoglobulin or plasma exchange , is an effective disease modifying treatment , reducing time to onset of recovery by 40-50 %. 3
Analgesia with simple agents , neuropathic pain relief or opioids may be required .
Outcome
Several weeks after IV immunogloblin treatment , Jack ’ s symptoms improve significantly and his left facial palsy resolves .
References on request from kate . kelso @ adg . com . au
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The patient ’ s MRI demonstrated fluid within the left mastoid sinus .
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ANSWER The answer is c . Phymatous rosacea , derived from the Greek word “ phyma ” meaning growth , is a chronic inflammatory condition characterised by tissue hypertrophy , sebaceous gland hyperplasia and dilated hair follicles . It most commonly affects the nose ( rhinophyma ), but can involve the ears ( otophyma ), chin ( gnatophyma ), eyelids ( blepharophyma ), and forehead ( metophyma ). 1 , 2 It is more common in men , contrasting with other subtypes of rosacea which mainly affect adult females aged 30-50 , particularly those of Northern European heritage with fair skin . 1 , 3 , 4
Rosacea is a chronic inflammatory disease characterised by recurrent facial flushing , and typically presents with erythema , telangiectasias , papules , or pustules of the face . 3 Patients often report facial burning or stinging during flares . 5 The diagnosis is clinical .
The papulopustular form of rosacea presents with facial papules and pustules which can appear similar to acne . Rosacea lacks comedones , which can help differentiate the condition from acne vulgaris . 3 A drug reaction would be more florid . Sebaceous hyperplasia is caused by enlarged sebaceous glands . It presents as one or multiple skin-coloured or yellow papules with central umbilication , commonly located on the face but can appear on other regions . Although sebaceous hyperplasia can co-occur with phymatous rosacea , in this case the features favour the latter .
Common rosacea triggers include extreme temperatures ( hot , cold ), UV exposure , spicy foods , alcohol , wind , exercise and stress . 3 The pathogenesis is not well characterised but a family history , environmental factors , micro-organisms ( demodex mites , Helicobacter pylori ), immune factors , and neurovascular dysregulation are thought to contribute . 3
There are several treatment options for phymatous rosacea . Topical treatment is not effective , but oral antibiotics ( doxycycline 40mg once daily ) or low-dose isotretinoin ( 0.3mg / kg daily ) may slow the progression of the disease . 4 , 6 Severe cases can be treated with ablative lasers to debulk excess tissue , or less commonly , scalpel and loop electrosurgical excision . 1 Other options include cryosurgery , ionising radiation , and energy-based lasers ( CO 2 laser , pulsed dye laser , or YAG lasers ). 6 Rosacea has also been reported to respond to secukinumab , a biologic that targets interleukin-17 . 7 Patients should also be counselled on the importance of and trigger avoidance , photoprotection and use of gentle cleansers and moisturisers to maintain skin hydration and barrier function .
References on request from kate . kelso @ adg . com . au
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