Claw-like hands revealed hidden nerve damage in woman with AIP
Researchers say the condition can be mistaken for a localized nerve injury
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A woman with acute intermittent porphyria (AIP) developed lasting nerve damage after repeated disease attacks, resulting in distinctive hand changes known as “claw hands,” according to a case report from Taiwan. In this condition, the fingers curve into a claw-like position due to damage affecting the nerves that control hand movement.
After she began treatment with Givlaari (givosiran), a therapy approved in the U.S. for acute hepatic porphyria, including AIP, her attacks stopped, and her symptoms eased.
Claw-like hand changes can mask an underlying condition
The researchers noted that claw-like hand changes can mimic ulnar nerve damage, a more localized nerve problem that affects finger movement. As a result, these hand changes may be mistaken for an isolated nerve injury rather than a sign of an underlying condition.
The authors emphasized that effective disease management should aim to control “both acute attacks and chronic complications.”
The case, “Claw hands in acute intermittent porphyria,” was published in Oxford Medical Case Reports.
AIP is the most common type of acute hepatic porphyria (AHP), a genetic condition caused by an impaired ability to produce heme. Heme is an essential molecule that helps red blood cells carry oxygen.
When heme production is disrupted, certain intermediate substances can build up to toxic levels. In AHP, this buildup primarily affects the nervous system. As a result, symptoms may include severe abdominal pain, muscle weakness, seizures, and, in some cases, paralysis.
Symptoms often appear suddenly during acute attacks, which can be severe and may last for days or even weeks before easing. Over time, repeated attacks may lead to nerve damage and lingering neurological symptoms, even between attacks.
Repeated attacks led to long-term nerve damage
In this case, doctors described a 48-year-old woman with AIP who developed long-term nerve complications after repeated disease attacks.
She experienced repeated episodes of cramp-like abdominal pain, along with gradually worsening weakness in her arms. The weakness began in her hands and slowly spread upward, affecting her right side more than her left.
Over time, she developed ongoing neurological symptoms, including involuntary movements, changes in sensation such as numbness or tingling, difficulty speaking, and episodes of paralysis affecting all four limbs.
Tests of nerve function showed widespread nerve damage affecting both movement and sensation. On examination, doctors noted reduced strength in certain hand muscles along with claw-like hand changes.
The woman was diagnosed with AIP in 2011. Three years later, she began preventive treatment with Panhematin (hemin for injection) and glucose. These treatments help restore heme levels and signal the body to slow heme production, reducing the buildup of toxic substances linked to AIP symptoms.
In 2018, she was enrolled in a Phase 3 ENVISION clinical trial (NCT03338816), which evaluated Givlaari in people with AIP. The therapy works by lowering the buildup of harmful substances involved in AIP attacks.
Treatment stopped attacks and eased nerve symptoms
After starting Givlaari, her annual attack rate dropped from about 24 episodes per year to zero. As the acute attacks stopped, her neurological symptoms also eased. Doctors observed improved finger movement and partial recovery of muscle loss in her hands.
The authors noted that hand changes like those seen in this case are often attributed to focal neuropathy, such as damage to the ulnar nerve.
“This case underscores the need to look beyond focal neuropathies,” the researchers wrote, stressing that claw hands might hinder recognition of an underlying disorder such as AIP.
The authors also emphasized that effective AIP care should focus not only on rapid treatment of acute attacks, but also on preventing long-term complications. In this context, the researchers noted that Givlaari can reduce the buildup of toxic substances that may contribute to nerve damage in porphyria.
