Neuro Short Notes Mohammad Kazmi

Stiff person syndrome


Stiff person syndrome (SPS) is a very rare autoimmune disorder that causes progressive rigidity and muscle spasms in the limbs and axial muscles. There are different types of SPS, including classic stiff person syndrome, stiff person variants, focal/segmental stiff person syndrome, jerky stiff person syndrome, progressive encephalomyelitis with rigidity and myoclonus (PERIM), stiff person syndrome plus ataxia, epilepsy, or other disorders, stiff leg/limb syndrome, paraneoplastic stiff person syndrome, and a subgroup associated with amphiphysin antibodies primarily in females with breast cancer and cervical spine region stiffness.

SPS can be associated with various neoplasia, including breast cancer, colorectal cancer, lung cancer, Hodgkin disease, and malignant thymoma. It can also be associated with autoimmune disorders such as diabetes mellitus type 1, Hashimoto thyroiditis, Graves disease, pernicious anemia, anti-NMDAR encephalitis, limbic encephalitis, refractory epilepsy, polyendocrine autoimmune syndrome, vitiligo, celiac disease, myasthenia gravis, autoimmune retinopathy, scleritis, and systemic lupus erythematosus (SLE).

The cause of SPS is an autoimmune condition that primarily affects the inhibitory mechanisms within the central nervous system. The associated antibodies include glutamic acid dehydrogenase (GAD 65), glycine, and amphiphysin.

The presentation of SPS includes episodic stiff and aching muscles starting in the axial muscles and progressing to the proximal limb muscles. It is characterized by restricted range of motion, slow voluntary movements resembling parkinsonism, muscle hypertrophy, postural abnormalities, and progressive interference with daily activities. Other symptoms include muscle tightness, aching, rigidity, and spasms, gait and balance difficulties, fatigue, persisting lumbar hyperlordosis, joint dislocations/fractures, abnormal postures, mask-like face, axial/truncal rigidity, muscle spasms, oculomotor disturbances, and fall risk.

Treatment options for SPS include benzodiazepines, baclofen, tizanidine, or gabapentin. IV immunoglobulin (IVIG) or rituximab may also be used. Other medications such as Levetiracetam, gabapentin, pregabalin, Tiagabine, valproate, Vegabatrin, Tizanidine, Danteolene, Propafol, and Botox can be considered.

Shingles affects 1 in 3 Americans, is more likely if you've had chickenpox. Aging increases the risk, with potential complications like postherpetic neuralgia, pneumonia or death. Early signs include pain and fever, rash typically appears on one side/one Dermatome. Shingrix for prevention, and antiviral medications can reduce severity. Start the treatment within 72 hours with Acyclovir, Valcyclovir or Famcyclovir  for seven days or so. PHN, lasting over three months, is a common complication in 10-50%. Corticosteroids aren't routinely recommended. If you're over 50 and had chickenpox, your immune system decline puts you at risk. Testing is done with the PCR with swabs of the lesions, CSF, tissue, bronchial  lavage, vitreal, aspirate, corneal swab. What is
Shingrix is over 90% effective and administered in two doses. Receiving both shots 2-6 months apart is crucial, and the CDC recommends it over ZOSTAVAX. Not recommended in pregnant or nursing. You can get shingles at zero co-pay .

A quick review of Mediterranean diet

Breast syndrome push the reversible encephalopathy

Intracranial hypertension, pseudotumor cerebri causes and treatment. A quick review on the subject.

Treatment of Bell’s-palsy