The “stiff-person syndrome” – what is it, and how to deal with it

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Dyah Tunjungsari
dr. Dyah Tunjungsari, Sp.N, Subsp.NGD(K). (Source: PONDOK INDAH HOSPITAL)

Jakarta, IO – The “stiff-person syndrome” is a rare neurological illness marked by muscle stiffness and repeated episodes of muscular spasms. This “stiffness” characteristically does not hit frequently moving parts of the body, but oddly enough, around the torso area: neck, chest, stomach, hips, and back – before finally spreading to the limbs. Sufferers report a difficulty of standing up and/or walking in certain circumstances, such as going up and down the stairs, because this process requires sufficiently dynamic movement of these very areas. 

“The stiff-person syndrome is a rare autoimmune disorder that attacks the central nervous system, thus causing stiffness, spasms, and cramps of the muscles. There is no proven theory of its causes yet, but most researchers suspect that it is because the body misreads the signal issued by the nerves as coming from ‘dangerous foreign bodies’, and thus ordered the immune system to attack it. The syndrome is generally suffered by young adults aged 30 to 40 years old,” reported dr. Dyah Tunjungsari, Sp.N, Subsp.NGD(K), neurology specialist sub-specializing in neurodegenerative diseases from Pondok Indah Hospital, Jakarta, in the “Stiff-Person Syndrome” live IG session held last Friday (13/01/2023). 

The stiff-person syndrome may be triggered by malignant conditions such as cancer, or by other autoimmune disorders. The severity level and progressivity speed of the syndrome differ among individuals. “In the early stages, it comes and goes without any clear triggers, and it may progress for the worse. Patients would feel extreme pain because repeated muscular spasms and become much more sensitive towards various stimulus, including physical touches and loud noises. In a severe stage, it causes spinal abnormalities, including permanent bending (kyphosis) or even (hyperlordosis),” dr. Dyah said. 

Not the Same as Parkinson’s 

Like with any other disease, early detection is essential for stiff-person syndrome sufferers. Early administration of treatment procedures lowers the risk of both disability and recurrence. Diagnosis of the syndrome is established using comprehensive interview, complete physical and blood tests, and glutamic acid decarboxylase (“GAD”) testing. “The doctor will perform anamnesis to determine whether the stiffness occurs in the marrow, bone, muscle, or nerves. GAD is a protein produced internally. It is involved in the production of gamma-aminobutyrate (“GABA”) neurotransmitters, which degrades muscular functions and preventing muscles from relaxing. Stiff-person syndrome patients have very high levels of GAD antibodies. We treat them using the immunomodulator therapy intravenous immunoglobulin (“IVIG”), which suppresses the body’s immunity response,” dr. Dyah said. “Other modes of treatment are routine physical exercises and physiotherapy, prescribed according to the patient’s condition. These exercises must be performed regularly for them to be beneficial to the patient.” 

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Even though they both cause stiffness in the muscles, there are significant differences between Parkinson’s disease and the stiff-person syndrome. “Other than the fact that Parkinson patients tend to be older, the trouble in walking tend to occur in later stages, preceded by tremors in the moving parts of the body. Parkinson patients also talk differently after the onset of the disease – their voices tend to become softer, and their facial expression tends to become monotone and mask-like. Furthermore, in this disease, cerebral MRIs tend to discover brain abnormalities, including decreased brain volume,” dr. Dyah said. “As the stiff-person syndrome is closely related to the body’s immune system, please consult your doctor about consuming supplements to bolster your immunity if you suffer from this illness.” (est)