Jaw Clonus in Osmotic Demyelination Syndrome and Neuromyelitis Optica Spectrum Disorder.

 

 Jaw clonus is a fascinating but uncommon clinical sign that points to trigeminal nerve supranuclear lesions. It has already been linked to amyotrophic lateral sclerosis in one report. An index case of jaw clonus in a neuromyelitis optica spectrum disorder patient with osmotic demyelination syndrome and pseudobulbar palsy caused by pontine corticobulbar fiber involvement is presented here.

A series of rhythmic, involuntary muscle contractions of the temporalis, masseter, and medial pterygoid muscles at a frequency of 5–7 Hz in response to a sudden and sustained stretch stimulus is known as jaw clonus. It usually means that the upper motor neurons in the corticopontine tracts have been damaged. In patients with supranuclear sores of the trigeminal nerve (for example amyotrophic sidelong sclerosis [ALS] broad various sclerosis [MS], respective corticobulbar dead tissue with pseudobulbar paralysis) the jaw jerk is misrepresented what's more, clonus might be observed.

Osmotic demyelination condition (ODS)

Osmotic demyelination condition (ODS) is a life‑threatening demyelinating condition, which as a rule happens in the setting of fast rectification of extreme constant hyponatremia. Focal pontine myelinolysis (CPM) and extrapontine myelinolysis (EPM), a subset of ODS, most reliably gives elements of pseudobulbar paralysis and spastic quadriplegia brought about by demyelination of corticospinal and corticobulbar plots either inside the pons or extra‑pontine cerebrum tissue,respectively.

Neuromyelitis Optica

Neuromyelitis Optica range problems (NMOSD, already known as Devic infection or neuromyelitis optica [NMO]) are provocative issues of the focal sensory system described by extreme, immune‑mediated demyelination and axonal harm overwhelmingly focusing on optic nerves and spinal line. As a result of severe vomiting brought on by involvement of the lower brainstem, NMO may result in hyponatremia. As a result, ODS continues to be a real possibility in NMO.

An unusual case of NMO complicated by subsequent ODS that presented with jaw clonus due to pseudobulbar palsy is the subject of this paper.

 A 40-year-old male driver with a diagnosis of "unarousable coma with hyponatremia". His medical history revealed that he had a sudden onset of vomiting that lasted for three days and did not respond to oral antiemetics. He was taken from his own vehicle in a tonic-clonic manner and found unconscious on the fourth day. He was found to have hyponatremia (Na 112 mEq/L) following initial resuscitation and a hypertonic (3 percent) saline infusion was attempted to rectify the situation.At first, he showed some improvement in cognizance and whined of decreased vision in the two eyes yet slowly over the course of the following couple of hours his sensorium further weakened and he was soon incapable to move his appendages. On the fifth day of his illness, he was brought to our attention early in the morning. Dilated pupils with a sluggish response to light, relative afferent papillary defect (RAPD), difficulty in deglutition, nasal regurgitation of liquids associated with cough, and flaccid symmetric quadriplegia were all found during the neurological examination. Except for jaw jerk and bilateral extensor plantar, none of the reflexes were present. Jaw jerk delighted jaw clonus.

Except for mild hyponatremia (Na  128 mEq/L), the initial laboratory tests were normal. Attractive reverberation imaging (X-ray) of the mind showed central adjusted power injuries, hyperintense on T2, fluid‑attenuated reversal recuperation (Pizazz), diffusion‑weighted picture (DWI) and hypo‑ to isointense on evident dissemination coefficient (ADC) with gentle inconsistent difference improvement at pons, both center cerebellar peduncles, crus of midbrain and two-sided basal ganglia . A bilateral retrobulbar intraorbital optic nerve demyelination was detected by MRI of the orbit. A visual evoked potential (VEP) test revealed normal P100-N70 wave amplitudes and long P100-N70 wave latencies, pointing to demyelinating bilateral retino-optic pathway dysfunction. Infectious processes and autoimmune encephalitis were ruled out during a CSF study using paired sera. CSF anti‑NMO/aquaporin 4 (AQP4) returned negative yet serum anti‑NMO immunizer was found to be positive in high titers alongside a couple oligoclonal groups. MR spine, MR angiography and DSA were typical.

Image 1: X-ray mind uncovers central modified signal power injuries hyper on T2 (an and f), Style (b), DWI and e) at pons, both center cerebellar

peduncles and crus of the midbrain and reciprocal basal ganglia. At the pons, contrast imaging reveals sporadic enhancement (d).

We hypothesized that NMO flare-up caused the intractable vomiting due to brainstem affection without radiological involvement of area postrema and hypothalamus (considering that 1.5 T MR may not always record the subtle signal changes) on the basis of classic clinical manifestations, lesions in MRI brain, and AQP4 positivity. However, concomitant ODS was strongly suggested by a history of rapid hyponatremia correction, symmetrical pontine and extrapontine signal changes, and little contrast enhancement. The patient was promptly treated with 5 days of intravenous methylprednisolone at a dose of 1 gm/day, followed by oral corticosteroids at a dose of 1 mg/kg and IVIG. Until his serum sodium level returned to normal after five days, the patient was treated for hypovolemic hyponatremia with very slow sodium replenishment and fluid resuscitation. His vision improved as a result of IVIG, his emotional lability began to decrease, and after six weeks, he was able to correctly identify colors. Appendage power likewise gotten to the next level step by step and profound ligament reflexes returned. To prevent further relapses, 50 mg of azathioprine were started twice daily.

The destruction of AQP4 channels in astrocytes in autopsy specimens of lesions of ODS has been established.

There are at least two reasonably possible mechanisms by which ODS can occur in NMO: autoantibodies targeting AQP4 channels have been the etiopathogenic mechanism for both NMO and ODS. 

a) the co-occurrence of ODS and NMO, which is caused by autoantibodies against the AQP4 water channel causing immune-mediated astrocyte dysfunction in both NMO and ODS; 

b) NMO leading to metabolic perturbation, a rapid change in serum osmoles, and hyponatremia due to either severe persistent and intractable vomiting caused by area postrema involvement (hypovolemic hyponatremia) or the syndrome of inappropriate anti On their own or while being treated, either of these can cause oligodendrocyte damage and, as a result, demyelination that leads to ODS. ODS and NMO have also been mentioned, albeit infrequently. 

Jaw clonus, a fascinating neurological sign, is rarely mentioned in the medical literature. In 1886, Beevor wrote about a case of amyotrophic lateral sclerosis with clonus of the lower jaw. Since then, only a few cases of jaw clonus in people with ALS have been reported. In this rare case, which is probably the index one, a patient with NMO had jaw clonus and then had ODS. The possible cause was pseudobulbar palsy because pontine corticobulbar fibers became  Anti-AQP4 positive individuals, whether or not they also have opticospinal involvement, may first present with hyponatremia.

Acute diencephalic or brainstem syndrome presenting NMOSD patients make up this group. EPM and CPM, two rather severe osmotic demyelination syndromes, can occur when hyponatremia is treated. It is common knowledge that ODS exhibits characteristics of pseudobulbar palsy.

However, despite the fact that jaw clonus is a manifestation of pseudobulbar palsy, it is a clinically extremely uncommon finding, and such presentations of NMOSD have not previously been documented in the literature. Jaw clonus happens because of respective pyramidal lot association is fundamentally found in MS, ALS, respective stroke and mind stem demyelination. Although it is uncommon, this finding is highly pathognomonic for suspicion of the aforementioned conditions and can easily be extrapolated to the primary care level with a straightforward jaw jerk examination and prompt referral to the higher center.

is a fascinating but uncommon clinical sign that points to trigeminal nerve supranuclear lesions. It has already been linked to amyotrophic lateral sclerosis in one report. An index case of jaw clonus in a neuromyelitis optica spectrum disorder patient with osmotic demyelination syndrome and pseudobulbar palsy caused by pontine corticobulbar fiber involvement is presented here.

A series of rhythmic, involuntary muscle contractions of the temporalis, masseter, and medial pterygoid muscles at a frequency of 5–7 Hz in response to a sudden and sustained stretch stimulus is known as jaw clonus. It usually means that the upper motor neurons in the corticopontine tracts have been damaged. In patients with supranuclear sores of the trigeminal nerve (for example amyotrophic sidelong sclerosis [ALS] broad various sclerosis [MS], respective corticobulbar dead tissue with pseudobulbar paralysis) the jaw jerk is misrepresented what's more, clonus might be observed.

Osmotic demyelination condition (ODS)

Osmotic demyelination condition (ODS) is a life‑threatening demyelinating condition, which as a rule happens in the setting of fast rectification of extreme constant hyponatremia. Focal pontine myelinolysis (CPM) and extrapontine myelinolysis (EPM), a subset of ODS, most reliably gives elements of pseudobulbar paralysis and spastic quadriplegia brought about by demyelination of corticospinal and corticobulbar plots either inside the pons or extra‑pontine cerebrum tissue,respectively.

Neuromyelitis Optica

Neuromyelitis Optica range problems (NMOSD, already known as Devic infection or neuromyelitis optica [NMO]) are provocative issues of the focal sensory system described by extreme, immune‑mediated demyelination and axonal harm overwhelmingly focusing on optic nerves and spinal line. As a result of severe vomiting brought on by involvement of the lower brainstem, NMO may result in hyponatremia. As a result, ODS continues to be a real possibility in NMO.

An unusual case of NMO complicated by subsequent ODS that presented with jaw clonus due to pseudobulbar palsy is the subject of this paper.

 A 40-year-old male driver with a diagnosis of "unarousable coma with hyponatremia". His medical history revealed that he had a sudden onset of vomiting that lasted for three days and did not respond to oral antiemetics. He was taken from his own vehicle in a tonic-clonic manner and found unconscious on the fourth day. He was found to have hyponatremia (Na 112 mEq/L) following initial resuscitation and a hypertonic (3 percent) saline infusion was attempted to rectify the situation.At first, he showed some improvement in cognizance and whined of decreased vision in the two eyes yet slowly over the course of the following couple of hours his sensorium further weakened and he was soon incapable to move his appendages. On the fifth day of his illness, he was brought to our attention early in the morning. Dilated pupils with a sluggish response to light, relative afferent papillary defect (RAPD), difficulty in deglutition, nasal regurgitation of liquids associated with cough, and flaccid symmetric quadriplegia were all found during the neurological examination. Except for jaw jerk and bilateral extensor plantar, none of the reflexes were present. Jaw jerk delighted jaw clonus.

Except for mild hyponatremia (Na  128 mEq/L), the initial laboratory tests were normal. Attractive reverberation imaging (X-ray) of the mind showed central adjusted power injuries, hyperintense on T2, fluid‑attenuated reversal recuperation (Pizazz), diffusion‑weighted picture (DWI) and hypo‑ to isointense on evident dissemination coefficient (ADC) with gentle inconsistent difference improvement at pons, both center cerebellar peduncles, crus of midbrain and two-sided basal ganglia . A bilateral retrobulbar intraorbital optic nerve demyelination was detected by MRI of the orbit. A visual evoked potential (VEP) test revealed normal P100-N70 wave amplitudes and long P100-N70 wave latencies, pointing to demyelinating bilateral retino-optic pathway dysfunction. Infectious processes and autoimmune encephalitis were ruled out during a CSF study using paired sera. CSF anti‑NMO/aquaporin 4 (AQP4) returned negative yet serum anti‑NMO immunizer was found to be positive in high titers alongside a couple oligoclonal groups. MR spine, MR angiography and DSA were typical.

Image 1: X-ray mind uncovers central modified signal power injuries hyper on T2 (an and f), Style (b), DWI and e) at pons, both center cerebellar

peduncles and crus of the midbrain and reciprocal basal ganglia. At the pons, contrast imaging reveals sporadic enhancement (d).

We hypothesized that NMO flare-up caused the intractable vomiting due to brainstem affection without radiological involvement of area postrema and hypothalamus (considering that 1.5 T MR may not always record the subtle signal changes) on the basis of classic clinical manifestations, lesions in MRI brain, and AQP4 positivity. However, concomitant ODS was strongly suggested by a history of rapid hyponatremia correction, symmetrical pontine and extrapontine signal changes, and little contrast enhancement. The patient was promptly treated with 5 days of intravenous methylprednisolone at a dose of 1 gm/day, followed by oral corticosteroids at a dose of 1 mg/kg and IVIG. Until his serum sodium level returned to normal after five days, the patient was treated for hypovolemic hyponatremia with very slow sodium replenishment and fluid resuscitation. His vision improved as a result of IVIG, his emotional lability began to decrease, and after six weeks, he was able to correctly identify colors. Appendage power likewise gotten to the next level step by step and profound ligament reflexes returned. To prevent further relapses, 50 mg of azathioprine were started twice daily.

The destruction of AQP4 channels in astrocytes in autopsy specimens of lesions of ODS has been established.

There are at least two reasonably possible mechanisms by which ODS can occur in NMO: autoantibodies targeting AQP4 channels have been the etiopathogenic mechanism for both NMO and ODS. 

a) the co-occurrence of ODS and NMO, which is caused by autoantibodies against the AQP4 water channel causing immune-mediated astrocyte dysfunction in both NMO and ODS; 

b) NMO leading to metabolic perturbation, a rapid change in serum osmoles, and hyponatremia due to either severe persistent and intractable vomiting caused by area postrema involvement (hypovolemic hyponatremia) or the syndrome of inappropriate anti On their own or while being treated, either of these can cause oligodendrocyte damage and, as a result, demyelination that leads to ODS. ODS and NMO have also been mentioned, albeit infrequently. 

Jaw clonus, a fascinating neurological sign, is rarely mentioned in the medical literature. In 1886, Beevor wrote about a case of amyotrophic lateral sclerosis with clonus of the lower jaw. Since then, only a few cases of jaw clonus in people with ALS have been reported. In this rare case, which is probably the index one, a patient with NMO had jaw clonus and then had ODS. The possible cause was pseudobulbar palsy because pontine corticobulbar fibers became  Anti-AQP4 positive individuals, whether or not they also have opticospinal involvement, may first present with hyponatremia.

Acute diencephalic or brainstem syndrome presenting NMOSD patients make up this group. EPM and CPM, two rather severe osmotic demyelination syndromes, can occur when hyponatremia is treated. It is common knowledge that ODS exhibits characteristics of pseudobulbar palsy.

However, despite the fact that jaw clonus is a manifestation of pseudobulbar palsy, it is a clinically extremely uncommon finding, and such presentations of NMOSD have not previously been documented in the literature. Jaw clonus happens because of respective pyramidal lot association is fundamentally found in MS, ALS, respective stroke and mind stem demyelination. Although it is uncommon, this finding is highly pathognomonic for suspicion of the aforementioned conditions and can easily be extrapolated to the primary care level with a straightforward jaw jerk examination and prompt referral to the higher center.