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ABSTRACT
Stiff person syndrome is a highly disabling, progressive autoimmune disorder of the central
nervous system characterized by muscle rigidity and spasms. Stiff person syndrome is rare, but is
believed to be under diagnosed with only 14 cases been reported among a 1.7 billion population
in South Asia.
INTRODUCTION
Stiff person syndrome (SPS), also known as stiff man syndrome (SMS) is a
rare neurologic disorder of unclear cause characterized by progressive rigidity and stiffness. It is
unique among neurologic diagnoses because of its lack of significant similarity to any other
neurologic diseases. Although rare, once observed it is quite unforgettable. SPS occurs in about
one in a million people and is most commonly found in middle-aged people. Possibly the closest
related disease is tetanus because both conditions affect peripheral inhibition via central
mechanisms and both conditions inhibit central gamma-aminobutyric acid (GABA) systems.
In 1956, Moersch and Woltmann, who also coined the term stiff man syndrome, first clearly
described stiff person syndrome as a neurologic clinical entity at the Mayo Clinic. The eponym
for this syndrome, Moersch-Woltmann syndrome, is one of the few instances in which the
eponym may be the most inclusive and at the same time the most appropriately limiting name for
the disease. Benzodiazepine-class drugs are the most common treatment; they are used for
symptom relief from stiffness.
Neuromyotonia (NMT), also known as Isaacs Syndrome and Isaacs-Merton syndrome,
is a form of peripheral nerve hyperexcitability that causes spontaneous muscular activity
resulting from repetitive motor unit action potentials of peripheral origin. Prevalence is unknown
but 100–200 cases have been reported so far.
NMT is a diverse disorder. As a result of muscular hyperactivity, patients may present with
muscle cramps, stiffness, myotonia-like symptoms (slow relaxation), associated walking
difficulties, hyperhidrosis (excessive sweating), myokymia, fasciculations (muscle twitching),
fatigue, exercise intolerance, myoclonic jerks and other related symptoms. The symptoms
(especially the stiffness and fasciculations) are most prominent in the calves, legs, trunk, and
sometimes the face and neck, but can also affect other body parts. NMT symptoms may fluctuate
in severity and frequency. Symptoms range from mere inconvenience to debilitating. At least a
third of people also experience sensory symptoms.
CASE REPORT
During my clinical posting in abc hospital. In neurological ward I observed a very rare case
diagnosed as neuromyotonia v/s stiff-man syndrome .Where the client’s both legs (from
sacrum to toes) are stiff and rigid, client is
 unable to bend his legs
 not able to lean forward
 not able to sit
 experiencing pain over different parts of the body
 unable to put extended foot steps
 seizures are present
 hypocalemic(s.calcium 5)
But Client is able to walk, with an abnormal gait .Client is experiencing these symptoms
from past 10 years after which he had a bike accident, for which he had not taken any
treatment.
X ray lumbar sacral spine result says mild diffuse ostiopenia, distal joint arthropathy seen
in multiple levels and calcification of ligaments attached to lesser trochander of femur.
Endocrinology references are given for decreased parathyroid levels (4.8pg/ml).Patient
was on Tab shelcal CT, neurobion forte, Valium, Gardinal and ultracet.
GENERAL DISCUSSION
Stiff-person syndrome (SPS) is a rare acquired neurological disorder characterized by
progressive muscle stiffness (rigidity) and repeated episodes of painful muscle spasms. Muscular
rigidity often fluctuates (i.e., grows worse and then improves) and usually occurs along with the
muscle spasms. Spasms may occur randomly or be triggered by a variety of different events
including a sudden noise or light physical contact. In most cases, other neurological signs or
symptoms do not occur. The severity and progression of SPS varies from one person to another.
If left untreated, SPS can potentially progress to cause difficulty walking and significantly
impact a person's ability to perform routine, daily tasks. Although the exact cause of SPS is
unknown, it is believed to be an autoimmune disorder and sometimes occurs along with other
autoimmune disorders.
Acquired neuromyotonia is an inflammatory disorder characterized by abnormal nerve impulses
from the peripheral nerves that result in continuous muscle fiber activity. Affected individuals
often experience progressive muscle stiffness and cramping especially in the hands and feet,
increased sweating, and delayed muscle relaxation. Symptoms may persist even during sleep or
under general anesthesia.
Stiff-person syndrome is considered by many researchers to be a spectrum of disease ranging
from the involvement of just one area of the body to a widespread, rapidly progressive form that
also includes involvement of the brain stem and spinal cord.
CONCLUSION
Stiff-person syndrome has been described in the medical literature under many different,
confusing names. Originally described as stiff-man syndrome, the name was changed to reflect
that the disorder can affect individuals of any age and of either gender. The Stiff man syndrome
is readily distinguished from the continuous muscle activity, spasm and cramps of
Isaac's syndrome and neuromyotonia which originate in the peripheral nervous system.
Fasciculations, myokymia, myotonia and complex repetitive discharges are characteristic of
these peripheral neuromuscular disorders.
REFERENCES
1. Nancy Theresa Rodgers. Stiff man syndrome[document on the internet]. Updated: May
30, 2017[cited2017 July 19 9pm].available from ;
http://emedicine.medscape.com/article/1172135-overview
2. Stiff man syndrome[document on the internet].[cited2017 July 19 9.30pm].available from
;https://rarediseases.org/rare-diseases/stiff-person-syndrome/
3. Neuromyotonia[document on the internet].[cited2017 July 19 10pm].available from ;
https://en.wikipedia.org/wiki/Neuromyotonia
4. Chang T, Lang B, Vincent A. Stiff person syndrome in South Asia.BMC research
notes[serial online] 2016 Oct 18[cited on 2017 July 20 at 9pm] ;9(1):468.available from
https://www.ncbi.nlm.nih.gov/pubmed/27756410.

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Rare case on stiff man syndrome versus neuromyotonia

  • 1. ABSTRACT Stiff person syndrome is a highly disabling, progressive autoimmune disorder of the central nervous system characterized by muscle rigidity and spasms. Stiff person syndrome is rare, but is believed to be under diagnosed with only 14 cases been reported among a 1.7 billion population in South Asia. INTRODUCTION Stiff person syndrome (SPS), also known as stiff man syndrome (SMS) is a rare neurologic disorder of unclear cause characterized by progressive rigidity and stiffness. It is unique among neurologic diagnoses because of its lack of significant similarity to any other neurologic diseases. Although rare, once observed it is quite unforgettable. SPS occurs in about one in a million people and is most commonly found in middle-aged people. Possibly the closest related disease is tetanus because both conditions affect peripheral inhibition via central mechanisms and both conditions inhibit central gamma-aminobutyric acid (GABA) systems. In 1956, Moersch and Woltmann, who also coined the term stiff man syndrome, first clearly described stiff person syndrome as a neurologic clinical entity at the Mayo Clinic. The eponym for this syndrome, Moersch-Woltmann syndrome, is one of the few instances in which the eponym may be the most inclusive and at the same time the most appropriately limiting name for the disease. Benzodiazepine-class drugs are the most common treatment; they are used for symptom relief from stiffness. Neuromyotonia (NMT), also known as Isaacs Syndrome and Isaacs-Merton syndrome, is a form of peripheral nerve hyperexcitability that causes spontaneous muscular activity resulting from repetitive motor unit action potentials of peripheral origin. Prevalence is unknown but 100–200 cases have been reported so far. NMT is a diverse disorder. As a result of muscular hyperactivity, patients may present with muscle cramps, stiffness, myotonia-like symptoms (slow relaxation), associated walking difficulties, hyperhidrosis (excessive sweating), myokymia, fasciculations (muscle twitching), fatigue, exercise intolerance, myoclonic jerks and other related symptoms. The symptoms (especially the stiffness and fasciculations) are most prominent in the calves, legs, trunk, and sometimes the face and neck, but can also affect other body parts. NMT symptoms may fluctuate in severity and frequency. Symptoms range from mere inconvenience to debilitating. At least a third of people also experience sensory symptoms.
  • 2. CASE REPORT During my clinical posting in abc hospital. In neurological ward I observed a very rare case diagnosed as neuromyotonia v/s stiff-man syndrome .Where the client’s both legs (from sacrum to toes) are stiff and rigid, client is  unable to bend his legs  not able to lean forward  not able to sit  experiencing pain over different parts of the body  unable to put extended foot steps  seizures are present  hypocalemic(s.calcium 5) But Client is able to walk, with an abnormal gait .Client is experiencing these symptoms from past 10 years after which he had a bike accident, for which he had not taken any treatment. X ray lumbar sacral spine result says mild diffuse ostiopenia, distal joint arthropathy seen in multiple levels and calcification of ligaments attached to lesser trochander of femur. Endocrinology references are given for decreased parathyroid levels (4.8pg/ml).Patient was on Tab shelcal CT, neurobion forte, Valium, Gardinal and ultracet. GENERAL DISCUSSION Stiff-person syndrome (SPS) is a rare acquired neurological disorder characterized by progressive muscle stiffness (rigidity) and repeated episodes of painful muscle spasms. Muscular rigidity often fluctuates (i.e., grows worse and then improves) and usually occurs along with the muscle spasms. Spasms may occur randomly or be triggered by a variety of different events including a sudden noise or light physical contact. In most cases, other neurological signs or symptoms do not occur. The severity and progression of SPS varies from one person to another. If left untreated, SPS can potentially progress to cause difficulty walking and significantly impact a person's ability to perform routine, daily tasks. Although the exact cause of SPS is unknown, it is believed to be an autoimmune disorder and sometimes occurs along with other autoimmune disorders. Acquired neuromyotonia is an inflammatory disorder characterized by abnormal nerve impulses from the peripheral nerves that result in continuous muscle fiber activity. Affected individuals often experience progressive muscle stiffness and cramping especially in the hands and feet, increased sweating, and delayed muscle relaxation. Symptoms may persist even during sleep or under general anesthesia.
  • 3. Stiff-person syndrome is considered by many researchers to be a spectrum of disease ranging from the involvement of just one area of the body to a widespread, rapidly progressive form that also includes involvement of the brain stem and spinal cord. CONCLUSION Stiff-person syndrome has been described in the medical literature under many different, confusing names. Originally described as stiff-man syndrome, the name was changed to reflect that the disorder can affect individuals of any age and of either gender. The Stiff man syndrome is readily distinguished from the continuous muscle activity, spasm and cramps of Isaac's syndrome and neuromyotonia which originate in the peripheral nervous system. Fasciculations, myokymia, myotonia and complex repetitive discharges are characteristic of these peripheral neuromuscular disorders. REFERENCES 1. Nancy Theresa Rodgers. Stiff man syndrome[document on the internet]. Updated: May 30, 2017[cited2017 July 19 9pm].available from ; http://emedicine.medscape.com/article/1172135-overview 2. Stiff man syndrome[document on the internet].[cited2017 July 19 9.30pm].available from ;https://rarediseases.org/rare-diseases/stiff-person-syndrome/ 3. Neuromyotonia[document on the internet].[cited2017 July 19 10pm].available from ; https://en.wikipedia.org/wiki/Neuromyotonia 4. Chang T, Lang B, Vincent A. Stiff person syndrome in South Asia.BMC research notes[serial online] 2016 Oct 18[cited on 2017 July 20 at 9pm] ;9(1):468.available from https://www.ncbi.nlm.nih.gov/pubmed/27756410.