Dr Shahjalal Khan

02/08/2025

Progressive encephalomyelitis with rigidity and myoclonus

Severe life threatening immune mediated disorder - mostly considered a variant of stiff person syndrome

Typically presents with remitting relapsing symptoms of SPS

Key clinical features are rigidity and prominent myoclonus
Often involves brainstem signs and dysautonomia.
Potential T cell and antibody mediated mechanism

Antibodies for diagnostics are
AntiGAD, antibody against glycine, DPPX

In summary treatment includes GABA- ergic medications like benzodiazepines for symptomatic relief.

Immunotherapy is the mainstay of treatment- corticosteroids, rituximab, IVIG and plasma exchange.

05/03/2025

Lamotrigine (LTG) is the best preventive therapy for SUNCT.

05/03/2025

Request HARNESS protocol for MRI brain in epilepsy patients for proper diagnostic approach neuro-radiologically .

10/12/2024

In one study Cogan lid twitch sign showed 99% specificity and 75% sensitivity for diagnosis of myasthenia gravis.

06/12/2024

Treatment is initiated with an oral formulation. Multiple oral options are available, including immediate-release (IR) tablets, controlled-release (CR) tablets, extended-release (ER) capsules, and disintegrating tablets.
Guidelines recommend IR as the preferential initial therapy in early PD over CR, because the latter has comparatively lower bioavailability and less predictable symptom relief.

CR is reserved for bedtime administration, because that timing helps reduce middle-of-the-night or early-morning “wearing off.”
ER capsules are given to patients who experience motor fluctuations despite dose/interval adjustments and use of adjunctive dopaminergic medications.

04/12/2024

Negative myoclonus (NM) is an unspecific motor disorder that can characterize a variety of neurological conditions. From the clinical point of view, NM appears as a shock-like involuntary jerky movement caused by a sudden, brief interruption of muscle activity. Asterixis is a type of NM that occurs typically in toxic-metabolic encephalopathies. NM of epileptic nature, or epileptic negative myoclonus (ENM), is defined as an interruption of tonic muscle activity, which is time-locked to an epileptic EEG abnormality, without evidence of an antecedent positive myoclonia in the agonist-antagonist muscles. ENM can be observed in idiopathic, cryptogenic, and symptomatic epileptic disorders. Pathophysiological hypotheses on the origin of NM involve subcortical as well as cortical mechanisms. Recent neuroimaging and neurophysiologic investigations, including intracerebral recordings and electrical stimulation procedures in epileptic patients, suggest the participation of premotor, primary motor, primary sensory, and supplementary motor areas in the genesis of NM. Polygraphic monitoring is essential for the diagnosis of NM, allowing the demonstration of brief interruptions of a tonic EMG activity, not preceded by a positive myoclonus in the agonist and antagonist muscles of the affected limb. Simultaneous EEG-EMG monitoring demonstrating the association of NM with an epileptic potential is consistent with the diagnosis of ENM. Evolution and prognosis of NM is mainly related to aetiology. In childhood idiopathic partial epilepsy, ENM can respond to some drugs (in particular, ethosuximide), whereas other medications (such as carbamazepine or phenytoin) have been reported to induce or worsen it.

05/11/2024

04/11/2024

Neurofibromatosis is an exam case for short stature.

Don't forget to look at the fundus for Lisch nodules.

29/07/2024

Extracranial cervical artery dissection: anticoagulation
Intracranial dissection: aspirin

12/07/2024

DAILY WARD CHALLENGE BY YOU
1. Don’t treat the disease; treat the patient as a whole with a very kind and empathic heart on the basis of your five medical ethics (autonomy, beneficence, non-maleficence, justice, and truth-telling).
2. Don’t treat the reports, treat the patient. E.g. urine routine examination shows 40 pus cells/HPF; don’t jump for the pus cells. Take a history from the patient, palpate his tummy, check his kidneys, check previous records, ask about LUTS, dip the urine; screen for urosepsis etc. Don’t just give the patient drip of juice of pharmaceuticals companies.
3. Manage the patient hairs to toes. You don’t only treat pneumonia; you must have to think about hair wash, cutting nails, a shower of the patient.
4. Medicine alone can’t cure the disease. Humans need good food, water, fresh air, love, warmth to living. So don’t just prescribe medicine. Ensure a good calorie diet; maintain good fluid balance, with a little bit of physio, a good walk, and a window in a room with good temperature. Don’t forget these are important for the health and soul.
5. Infection is deadly. So always maintain infection control policy, at least wash your hands before touching the noble person who is actually teaching you with the disease he/she got. Check for the entry ports (cannula, catheter, Ryle’s tube, PEG tube, CV catheter, etc.), check the patients’ bed, surroundings, his/her back (maybe there is sore; no one knows).
6. Control your tongue in front of your patient. Your tongue is a sharp razor that should be in your control regarding bad news. Don’t gossip with your fellows in front of the sickly.
7. Behave gently with your supporting staff. They are helping you with your job. You are the captain of the ship. You have to know every detail. But ask for help when you need it.

©DR MD SHAHJALAL KHAN; MRCP(UK); FCPS(MEDICINE)

11/07/2024

Symptoms checker series
For history taking during your clerking patient
*****
Scenario: Abnormal bleeding

Onset, course, duration, progression, aggravating factors- trauma, surgery, drugs, infections; relieving factors- avoidance; association- systemic feature + evidence of coagulation abnormality + evidence of CTD
Also try to assess- site + severity + mucosal bleeding + deep seated bleeding like- joint, muscle, abdomen, lungs
Description of the bleeding spot- petechiae, purpura- palpable or non-palpable, joint swelling, tummy distension
Family history of hemophilia, vWD, other coagulation factor deficiency, HHT, EDS
Personal history- alcohol intake very important
Concomitant disease- CLD, CKD, sepsis, pregnancy, TTP, ITP, HIV, malignancy
Etiological clues-
HHT- telangiectasia, epistaxis, hematemesis, melaena, headache, stroke, jaundice, hepatic bruit
EDS- skin laxity, hypermobile joint, bleeding, GI bleeding
ITP- prodrome, sore throat, RTI, CTD, HIV, pregnancy, lymphoma, drugs
Other thrombocytopenias- dengue, infection, marrow infiltration by leukaemia, lymphoma, aplastic anemia, CLD, CKD, TTP, drugs- heparin, vancomycin, antibiotics
Hemophilia- muscle bleeding, joint, abdominal
vWD- epistaxis, menorrhagia, GI bleeding, family history
liver failure, DIC- sepsis, pregnancy complication, Drugs- anti-coagulants, antiplatelets

11/07/2024

Symptoms checker series
During history taking

*****
Anemia with Low MCV
Symptoms of anemia- fatigue, weakness, dizziness, racing of heart during exertion
Symptoms of etiology-
Blood loss- menorrhagia(period details), blood loss from other orifices ( hematuria, epistaxis, hematemesis, melaena, hemoptysis)
Occult gastric or colore**al malignancy- altered bowel habit, weight loss, per re**al bleeding, tummy pain, discomfort, sense of incomplete evacuation
PUD- heartburn, nausea, upper chest pain related with food, drugs like aspirin, steroid, NSAID
IBD, diverticulitis, polyp – tummy pain, eye pain + rash, back pain, bloody diarrhea
Infection- hookworm + schistosomiasis- residenc, bare foot travel, swimming in pond
Chronic use of NSAID
Malabsorption- celiac disease- loose motion aggravated by foods like wheat, rye, barley, oats; back bain, easy bruise, sore tongue, gum bleeding
Previous gastric surgery- details history
HHA- ethnicity, history of consanguineous marriage between parents,
HHT- telangiectasia, pigmentation inside mouth, liver, GIT, lung, brain aneurysm or manifestation
Others history family history, dietary history for iron containing foods, chronic drug use