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Arjun G. Shah

@neurogenicpoet

Mar 17

Distraught parents bring their son to you. They tell you that he has been having weakness of his left hand since 1 year. With wasting. No sensory symptoms. They have been told, Motor Neurone Disease . You look at his hand and smile reassuringly. Why?

First things first, the demographics don't fit ALS. He's a young boy. Secondly, The disease seems quite restricted. Third, no signs of upper motor neuron disease. Finally, this OBLIQUE AMYOTRPOHY is classical and clinches a diagnosis of HIRAYAMA DISEASE

#MedTwitter #NeuroTwitter

Hirayama Disease typically affects young Asian males in their twenties. I have seen dozens of patients with Hirayama, never a girl (although case reports exist)
These boys tend to be lean and have long necks!
It is much more benign compared to ALS!

Read 13 tweets

Arjun G. Shah

@neurogenicpoet

Mar 14

You are again asked to see a patient with GBS. A young man in his 30's, does not seem perturbed by his acute onset of weakness of lower limbs. He says he has been through it on many occasions before. This particular bout was triggered after a hearty meal at a wedding. Thoughts?

An important differential to GBS are the Channelopathies. This gentleman had low levels of Potassium, and correction of the same completely reversed his symptoms! On evaluation, he was diagnosed with a Channelopathy
Simplified Approach

#Neurology

Hypokalaemia is notorious to cause neurological weakness often mimicking GBS. However clinical pointers for it are
- Recurrent Attacks
- Triggered by Alcohol/Carbs
- No Bulbar/ Facial Involvement
-Preserved reflexes (Can have areflexia!)
-Rapid (to come and go)
-Normal CSF

Read 16 tweets

Arjun G. Shah

@neurogenicpoet

Mar 9

Young boy w/ acute onset quadriparesis with areflexia, admitted to ICU with "GBS". You happen to glance at Urine Bag. something is off! The old urine is darker!? The patient is squealing with abdominal pain. Relatives tell you- seizures in the past
Thoughts?

#MedTwitter

This surely doesn't feel like a routine GBS. Young boy with seizures and abdominal pain, think of the Porphyrias!

Our patient probably has one of the acute Porphyrias. Confirmation of the type eluded us unfortunately since genetic analysis is expensive. Probably AIP

Neuro Porphyrias include
1. Acute Intermittent Porphyria (AD)
2. Hereditary Coproporphyria (AD)
3. Variegate Porphyria (AD)
4. ALA Dehydrates Porphyria

Read 11 tweets

Arjun G. Shah

@neurogenicpoet

Mar 6

2 brothers in their 30’s presented to us with progressive Cerebellar ataxia. On examination, there is slightly restricted vertical gaze.

Thoughts ?

#MedTwiter #NeuroTwitter #Neurology

One of the typical features of Niemann-Pick C disease is SUPRANUCLEAR VERTICAL GAZE PALSY

Ataxia, Dystonia Psychomotor Regression are common

GELASTIC CATAPLEXY is another common feature

The disease follows an Autosomal Recessive inheritance and unlike A/B, C can present in older ages with neurological features, without hepatosplenomegaly

Read 9 tweets

Arjun G. Shah

@neurogenicpoet

Dec 29, 2022

Resources to study #Neurology

Adams and Victor’s. By far my favourite textbook.
The book is more clinically oriented, coloured with anecdotes and mental models. Reading it feels like seeing a pt in the ward/OPD

#MedTwitter #neurotwitter

It has elements of philosophy, history and is written eloquently.
Whimsical, yet profound, it’s teachings stay with me. Added bonus, my Guru in Neurology finds mention in the text 🙃 I’d recommend this for #mbbs #md and #dm students

Bradley is the standard #textbook in #neurology. A great book, it’s more like #Harrison. Great for information, latest research and management. Essential for the DM #neurology candidate, but also useful for MD #internalmedicine

Read 11 tweets

Arjun G. Shah

@neurogenicpoet

Dec 27, 2022

@StanleyKubrick

A fascinating symptom in #neurology is #Alien Hand syndrome
If anyone has seen @StanleyKubrick ‘s great film Dr. Strangelove, you might have wondered what’s wrong with the titular character

His hand has a #mind of its own!!!

#cinema #neurotwitter #MedTwitter

Simplifying , for carrying out bimanual planned movements, we need a Supplementary Motor Cortex (SMA) (left frontal lobe). This communicates to the opposite side via the corpus callosum. Lesions in any will impair proper bimanual function and cause an “alien limb”#neuroanatomy

Frontal variant (left sma lesion) : Patient will have impulsive groping and difficulty releasing objects
Affects the dominant hand
Can be due to a #stroke, #degeneration

My patient impulsively grabs my hand (with his right hand) despite me telling him not to!
#neurotwitter