What is Stiff Person Syndrome and how this 52-year-old patient recovered with IVIG treatment

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A 52-year-old gentleman, who had been suffering from severe stiffness and difficulty walking for months, has shown a remarkable improvement following treatment with intravenous immunoglobulin (IVIG) therapy. After experiencing progressive tightness in his back and legs, the patient found it increasingly difficult to walk more than 200-300 meters without the onset of intense muscle stiffness, which led to him losing his balance and needing to catch hold of nearby walls for support.

Initially, the patient's condition raised concerns for possible spinal canal stenosis and vascular claudication, but after thorough testing, including MRI and Doppler scans, no such conditions were found. With the clear absence of clues in the investigations pertaining to common neurological and vascular issues, a clinical diagnosis of Stiff Person Syndrome (SPS) was made.

Stiff Person Syndrome is a rare neurological condition characterised by severe stiffness and muscle spasms, particularly in the back, legs, and arms. This condition often goes underdiagnosed, as its symptoms can resemble those of psychological conditions or other neurological diseases. However, in this case, a detailed assessment led to the initiation of IVIG therapy.

The results were immediate. Within just one day of receiving the first dose of IVIG, the patient showed significant improvement - reporting a 60% reduction in stiffness. After five days of treatment, the patient was able to walk for 15 minutes without experiencing the tightness and discomfort that had previously hindered his mobility.

Dr. Sheetal Goyal, consultant neurologist at Wockhardt Hospitals, Mumbai Central, said, "This case exemplifies the importance of clinical acumen in diagnosing rare conditions like Stiff Person Syndrome, which can easily be overlooked. Despite standard tests like MRI and Doppler coming back normal, we took a holistic approach to the patient's symptoms and were able to treat him effectively. The patient's quick recovery with IVIG therapy highlights the potential for successful outcomes even in rare neurological disorders."

Despite the absence of the typical autoimmune antibodies associated with Stiff Person Syndrome, the patient responded remarkably well to the IVIG treatment. The patient's quick recovery allowed him to resume his daily activities and return to work earlier than expected. He also expressed his gratitude in a letter, thanking the medical team for their swift diagnosis and treatment.

"The case highlights the importance of clinical acumen in diagnosing rare conditions, even when standard tests come back normal," said Dr. Goyal. "Stiff Person Syndrome is often overlooked or misdiagnosed, and this patient's rapid improvement is a testament to the effectiveness of IVIG in managing such rare conditions."

This case underscores the value of early intervention and the role of personalised treatment in addressing rare neurological disorders. As Stiff Person Syndrome remains a largely unrecognized condition, the healthcare community is encouraged to be vigilant and proactive when diagnosing patients with unexplained muscle stiffness.