marchiafava bignami syndrome

Sunday, June 6, 2021

A 38 year old male with chief complaints of forgetfulness and irrelevant talking.

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Name : D Arun Kumar

Roll. No. 046

Intern

Case history :

A 38 year old male patient, presented to the OPD with the chief complaints of  

1. forgetfulness since 3 months,  

2. irrelevant talk since 3 months, and 

3.gait disturbances since 2 months.

History of presenting illness :

Patient was apparently asymptomatic 3 years back when he developed pain abdomen and was admitted to various hospitals and was diagnosed with acute pancreatitis.

He was diagnosed with type 2 diabetes mellitus, (secondary to acute pancreatitis) 3 years back. Since lockdown, he has been consuming more than 1litre of whiskey per day. 

 3 months back he consumed only  alcohol without any food for one week, and One day morning, the attenders were unable to wake him up from sleep. He was taken to Mamatha medical College where he was diagnosed with alcoholic ketosis and was on treatment for 2 weeks . Since then , he has not been able to talk, he has not been able to respond to command. He has been unable to remember things and has not been able to do things by himself.

Past history:

 patient is a known case of type 2 diabetes mellitus since 3 years and was on medication. 

Personal history:

Married

Appetite:  normal 

Diet:  mixed 

Bowel and bladder habits :  regular

Sleep:  adequate

Addictions :   alcoholic since 8years , used to consume 1litre of whiskey per day; 

smoked 2-4 cigarettes daily since 1 year. 

Family history: not significant.

General examination: 

The examination was done after obtaining informed consent in a well lit room.

The patient was conscious, well oriented to persons, Not oriented to place and time. 

There was no pallor, icterus, clubbing, cyanosis, lymphadenopathy, and edema. 

Vitals :

Temperature : afebrile

Pulse rate : 70bpm

Respiratory rate : 20 cps

Blood pressure : 110/70 mmHg

SpO2 : 99% at room temperature. 

GRBS : 269 mg/dl

Cvs: 

S1, S2 heard 

Respiratory system: Bilateral air entry is present. 

Abdomen: 

Soft, No tenderness, no palpable mass. 

Central nervous system :

  Conscious, not oriented to time , place and person 

Speech: no response.

No signs of meningeal irritation ( no neck stiffness, kerning's sign negetive)

Pupils - NRSL

Rombergs sign - negetive

   

                                         Right.                    Left 

Tone- UL.                            N                          N

           LL.                            N                          N

Power- UL                         5/5                       5/5

              LL.                        5/5.                      5/5

Reflexes.        

             B.                             +2.                          2+

             T.                              +2.                          2+

             S.                              +2.                          2+

             K.                                -   Not    elicited   -

             A.                               +.                             +

             P.                       decreased.          decreased

Lobar function:

Frontal lobe : unable to perform problem solving, lack of insight.

Parietal lobe : able to perform series of motor activities.

Right and left orientation ---->positive

Finger recognition ---->positive

Visuo-spatial orientation ----> negetiv

Occipital lobe : able to recognise familiar faces. 

Investigations:

Hemogram-   

Normocytic normochromic 

Urine examination-

RFT -

UREA.                 :  14mg/dl

CREATININE.     :  0.8 mg/dl

PHOSPHORUS  :  4.6 mg/dl

SODIUM            :  138 mEq/L

POTASSIUM     :  4.6 mEq/L

CHLORIDE        :  99 mEq/L

LFT- 

AST : 16 IU/L

ALT : 10 IU/L

ALP : 180 IU/L

TOTAL PROTEINS : 6.5 mg/dl

ALBUMIN : 3.77 mg/dl

A/G RATIO : 1.38 

ECG-

Vent rate : 60BPM

PR interval : 138ms

QRS duration : 74ms

QT/QTc : 419/419ms

Avg RR : 996ms

P-R-T axes : 12  23  34

MRI

Mild diffusely thinned out Corpus callosum.

Normal grey/white matter differentiation

Nasal ganglia and thalami are normal

Cranio-vertebral and Cervico-medullary junctions are normal.

Sella, pituitary and parasellar regions are normal. 

Pituitary gland, pituitary stalk, and hypothalamus are normal. 

Provisional diagnosis:

Alcohol induced dementia 

K/c/o type 1 diabetes mellitus

K/c/o alcohol dependence syndrome. 

Treatment:

Inj. Thiamine 2amp in 100ml NS /IV/ TID

Inj. Human Mixtard S/C -- 

Tab. Haloperidol 165mg PO/OD

Tab. Pregabalin 75mg PO/MS 

Tab. Divalproate 100mg 

Tab. Memantine 10 mg 

Tab. Sprolit plus 1/2 tab OD